Myeloproliferative Neoplasms (MPN) are characterized by the overproduction of blood cells (red blood cells, white blood cells, and/or platelets) or collagen in the bone marrow. Often the MPN will be identified because of a blood test for another condition, as some recipients are asymptomatic. Common symptoms found in the array of myeloproliferative disorders include fatigue and the enlargement of the spleen (splenomegaly).

Question 259: What was the MPN subtype at diagnosis?

Indicate the MPN subtype at diagnosis and continue with question 262.

If the MPN subtype is “Myeloproliferative neoplasm (MPN), unclassifiable” continue with question 261. If the MPN subtype is “Systemic mastocytosis” continue with question 260.

Question 260: Specify systemic mastocytosis

Specify the systemic mastocytosis sub-type / variant and continue with question 262.
The diagnosis of systemic mastocytosis can be made when the major criterion and at least 1 minor criterion are present, or when >/= 3 minor criteria are present.

  • Major criterion: Multifocal dense infiltrates of mast cells (>/= 15 mast cells in aggregates) detected in sections of bone marrow and/or other extracutaneous organs(s).
  • Minor criteria:
  1. In biopsy sections of bone marrow or other extracutaneous organs, >25% of the mast cells in the infiltrate are spindle-shaped or have atypical morphology; or >25% of all mast cells in bone marrow aspirate smears are immature or atypical.
  2. Detection of an activating point mutation at codon 816 of KIT in the bone marrow, blood or another extracutaneous organ.
  3. Mast cells in bone marrow, blood or another extracutaneous organ express CD25, with or without CD2, in addition to normal mast cell markers.
  4. Serum total tryptase is persistently >20 ng/ml, unless there is an associated myeloid neoplasm, in which case this parameter is not valid.

The diagnostic criteria for the systemic mastocytosis sub-types/variants are as follows. Each sub-type/variant meets the general criteria for systemic mastocytosis with additional criteria for each.

  1. Indolent systemic mastocytosis: Low mast cell burden; no evidence of an associated hematologic neoplasm; skin lesions are almost invariably present; no “C” findings
  2. Smoldering systemic mastocytosis: >/=2 “B” findings and no “C” findings; high mast cell burden; no evidence of an associated hematologic neoplasm; does not meet criteria for mast cell leukemia
  3. Systemic mastocytosis with an associated hematologic neoplasm: Meets the criteria for an associated hematologic neoplasm (i.e., MDS, MPN,AML, lymphoma or another hematological neoplasm classified as a distinct entity in the WHO classification).
  4. Aggressive systemic mastocytosis: >/=1 “C” findings; does not meet the criteria for mast cell leukemia; skin lesions are usually absent.
  5. Mast Cell leukemia: Bone marrow biopsy shows diffuse infiltrate of atypical, immature mast cells; bone marrow aspirate smears show >/=20% mast cells. In classic cases, mast cells account for >/=10% of the peripheral blood WBC, but the aleukemic variant (in which mast cells account for <10%) is more common. Skin lesions are usually absent

“B” (burden of disease) and “C” (cytoreduction-requiring) findings in systemic mastocytosis.

“B” findings

  1. BM biopsy showing >30% infiltration by MC (focal, dense aggregates) and/or serum total tryptase level >200 ng/mL
  2. Signs of dysplasia or myeloproliferation, in non‐MC lineage(s), but insufficient criteria for definitive diagnosis of a hematopoietic neoplasm (AHNMD), with normal or slightly abnormal blood counts.
  3. Hepatomegaly without impairment of liver function, and/or palpable splenomegaly without hypersplenism, and/or lymphadenopathy on palpation or imaging.

“C” findings

  1. Bone marrow dysfunction manifested by one or more cytopenia(s) (ANC <1.0 × 109/L, Hgb <10 g/dL, or platelets <100 × 109/L), but no obvious non-mast cell hematopoietic malignancy.
  2. Palpable hepatomegaly with impairment of liver function, ascites and/or portal hypertension.
  3. Skeletal involvement with large osteolytic lesions and/or pathological fractures.
  4. Palpable splenomegaly with hypersplenism.
  5. Malabsorption with weight loss due to gastrointestinal mast cell infiltrates.

Question 261: Was documentation submitted to the CIBMTR (e.g. pathology report used for diagnosis)?

Indicate whether documentation for “myeloproliferative neoplasm, unclassifiable” was submitted to the CIBMTR (e.g., pathology report). For further instructions on how to attach documents in FormsNet3SM, refer to the Training Guide.

Questions 262: Did the recipient have constitutional symptoms (> 10% weight loss in six months, night sweats, unexplained fever higher than 37.5°C) in six months before diagnosis?

Indicate if constitutional symptoms were present at diagnosis. Constitutional symptoms are often called “B” symptoms and include unexplained fever greater than 38°C (100.4°F), night sweats, or unexplained weight loss in the six months prior to diagnosis. Indicate “yes” if any constitutional symptoms were present at or six months prior to diagnosis.

Indicate “no” if constitutional symptoms were not present at or prior to diagnosis. Indicate “unknown” if it is not possible to determine the presence or absence of constitutional symptoms at or six months prior to diagnosis.

Question 263: Date CBC drawn

These questions are intended to capture the laboratory studies performed at that diagnosis of MPN. Testing may be performed multiple times at diagnosis; report the most recent laboratory results performed prior to the start of first treatment of the primary disease for HCT. If the recipient’s MPN transformed, report the studies from the original diagnosis.

Report the date the sample was collected for testing and continue with question 265.

Questions 264-265: WBC

Indicate whether the white blood cell (WBC) count was “known” or “unknown” at diagnosis. If “known,” report the laboratory count and unit of measure documented on the laboratory report in question 265. If “unknown,” continue with question 266.

Questions 266-267: Neutrophils

Indicate whether the neutrophil percentage in the blood was “known” or “unknown” at diagnosis. If “known,” report the value documented on the laboratory report in question 268. If “unknown,” continue with question 269.

Questions 268-269: Blasts in blood

Indicate whether the percent blasts in the peripheral blood is “known” or “unknown” at the time of diagnosis.

If “known,” report the laboratory value in question 269. Note, blasts are not typically found in the peripheral blood. If blasts are not noted on the differential, you can still indicate “known” and report “0%” in question 269.

If the percent blasts in blood at diagnosis is not known, report “unknown” and go to question 270.

Questions 270-271: Hemoglobin

Indicate whether the hemoglobin was “known” or “unknown” at diagnosis. If “known,” report the laboratory value and unit of measure documented on the laboratory report in question 271. If “unknown,” continue with question 273.

Question 272: Was RBC transfused ≤ 30 days before the CBC sample date?

Transfusions temporarily increase the red blood cell count. It is important to distinguish between a recipient whose body is creating these cells and a recipient who requires transfusions to support the counts.

Indicate if red blood cells were transfused less than or equal to 30 days prior to the date reported in question 263.

Questions 273-274: Platelets

Indicate whether the platelet count was “known” or “unknown” at diagnosis. If “known,” report the laboratory count and unit of measure documented on the laboratory report in question 274. If “unknown,” continue with question 276.

Question 275: Were platelets transfused ≤ 7 days before the CBC sample date?

Transfusions temporarily increase the platelet count. It is important to distinguish between a recipient whose body is creating the platelets and a recipient who requires transfusions to support the counts.

Indicate if platelets were transfused less than or equal to 7 days prior to the date reported in question 263.

Questions 276-277: Blasts in bone marrow

Indicate whether the percentage of blasts in the bone marrow was “known” or “unknown” at the diagnosis. If “known,” report the percentage documented on the laboratory report in question 277. If “unknown,” continue with question 278.

Question 278-287: Were tests for driver mutations performed?

Testing for driver mutations may be performed by different methods including next generation sequencing (NGS), polymerase chain reaction (PCR), microarray, and fluorescence in situ hybridization (FISH). If testing was performed by any / all of these methods at diagnosis, report “yes” and report the results for the most recent test(s) in questions 279-287.

If testing for driver mutations were not performed or is unknown, report “no” or “unknown” and continue with question 289.

Question 288: Was documentation submitted to the CIBMTR (e.g. pathology report used for diagnosis)?

Indicate whether documentation was submitted to the CIBMTR (e.g., pathology report). For further instructions on how to attach documents in FormsNet3SM, refer to the Training Guide.

Question 289: Were cytogenetics tested (karyotyping or FISH)?

Cytogenetics is the study of chromosomes. Cytogenetic assessment involves testing blood or bone marrow for the presence of known chromosomal abnormalities that reflect the recipient’s disease. Testing methods include conventional chromosome analysis (karyotyping) or fluorescence in situ hybridization (FISH). For more information about cytogenetic testing and terminology, see Appendix C, Cytogenetic Assessments.

Indicate if cytogenetic studies were obtained at diagnosis. If cytogenetic studies were obtained, select “yes” and continue with question 290.

If no cytogenetic studies were obtained or it is unknown if chromosome studies were performed, select “no” or “unknown” and continue with question 306.

Question 290: Were cytogenetics tested via FISH?

Indicate if FISH studies were performed at diagnosis. If FISH studies were performed, report “yes” and continue with question 291.

If FISH studies were not performed at diagnosis, report “no” and continue question 298. Examples include: no FISH study performed, or FISH sample was inadequate. See Appendix C, Cytogenetic Assessments, for assistance interpreting FISH results.

Report chromosomal microarrays / chromosomal genomic arrays as FISH assessments.

Question 291: Sample source:

Indicate if the sample was from “bone marrow” or from “peripheral blood” and continue with question 292. If multiple sources were used to test FISH, the most preferred sample is the bone marrow.

Question 292: Results of tests:

If FISH assessments identified abnormalities, indicate “abnormalities identified” and continue with question 293.

If FISH assessments were unremarkable, indicate “no abnormalities” identified, continue with question 297.

Question 293-296: Specify cytogenetic abnormalities (FISH)

Report the International System for Human Cytogenetic Nomenclature (ISCN) compatible string, if applicable, in question 293, then continue with question 294.

Report the number of abnormalities detected by FISH at diagnosis in question 294, then select all abnormalities detected in question 295.

If a clonal abnormality is detected, but not listed as an option in question 296, select “other abnormality” and specify the abnormality in question 296. If multiple “other abnormalities” were detected, report “see attachment” in question 296 and attach the final report(s) for any other abnormalities detected. For further instructions on how to attach documents in FormsNet3 SM, refer to the Training Guide.

Question 297: Was documentation submitted to the CIBMTR?

Indicate whether documentation was submitted to the CIBMTR (e.g., pathology report, FISH report). For further instructions on how to attach documents in FormsNet3 SM, refer to the Training Guide.

Question 298: Were cytogenetics tested via karyotyping?

Indicate if karyotyping was performed at diagnosis. If karyotyping was performed, report “yes” and continue with question 299.

If karyotyping was not performed at diagnosis, report “no” and continue with question 307. Examples of this include: karyotyping was not performed, or karyotyping sample was inadequate.

Question 299: Sample source:

Indicate if the sample was from “bone marrow” or from “peripheral blood” and continue with question 300. If multiple sources were used for karyotyping analyses, the most preferred sample is the bone marrow.

Question 300: Results of tests:

If karyotyping assessments identified abnormalities, indicate “abnormalities identified” and continue with question 301.

If karyotyping assessments yielded no evaluable metaphases or there were no abnormalities identified, indicate such and continue with question 305.

Question 301-304: Specify cytogenetic abnormalities (karyotyping)

Report the International System for Human Cytogenetic Nomenclature (ISCN) compatible string, if applicable, in question 301, then continue with question 302.

Report the number of abnormalities detected by karyotyping at diagnosis in question 302. After indicating the number of abnormalities in question 302, select all abnormalities detected in question 303.

If a clonal abnormality is detected, but not listed as an option in question 303, select “other abnormality” and specify the abnormality in question 304. If multiple “other abnormalities” were detected, report “see attachment” in question 304 and attach the final report(s) for any other abnormalities detected. For further instructions on how to attach documents in FormsNet3 SM, refer to the Training Guide.

Question 305: Was documentation submitted to the CIBMTR?

Indicate whether documentation was submitted to the CIBMTR (e.g., karyotype report). For further instructions on how to attach documents in FormsNet3 SM, refer to the Training Guide.

Question 306: Did the recipient progress or transform to a different MPN subtype or AML between diagnosis and the start of the preparative regimen / infusion?

MPN subtypes may also transform/progress from one into another. Indicate if the recipient’s disease progressed to AML or transformed into a different MPN subtype between initial diagnosis and the start of the preparative regimen / infusion. Progression to AML is defined by an increase in blood or bone marrow blasts equal to or greater than 20%.

If the recipient’s disease did transform or progress, select “yes” and continue with question 307. If there was no documented transformation or progression, select “no” and continue with question 310.

Question 307: Specify the MDS subtype after transformation:

Indicate the recipient’s current MPN subtype after transformation. If the recipient experienced more than one transformation after diagnosis, report the most recent subtype. For a list of MPN subtypes and their diagnostic criteria, see Appendix H.

If the disease transformed to AML, continue with question 309.

For all other progressions or transformations, continue with question 308.

Question 308: Specify the date of the most recent transformation:

Report the date of assessment that determined the most recent disease transformation (i.e., if there were multiple transformations, report the most recent). Report the date of the pathological evaluation (e.g., bone marrow) or blood/serum assessment (e.g., CBC, peripheral blood smear). Enter the date the sample was collected for pathological and laboratory evaluations.

If the exact date is not known, use the process for reporting partial or unknown dates as described in General Instructions, Guidelines for Completing Forms.

Question 309: Date of MDS Diagnosis

If the recipient’s MPN transformed to AML prior to HCT, report the date of diagnosis of MPN. If the exact date is not known, use the process for reporting partial or unknown dates as described in General Instructions, Guidelines for Completing Forms.

Ensure the date of diagnosis for AML has been reported in question 1, AML is reported as the primary disease for HCT in question 2, and the AML section of the Disease Classification Form has been completed. Go to the signature line.

Question 310: Specify transfusion dependence at the last evaluation prior to the start of the preparative regimen / infusion:

Indicate the transfusion dependence for the recipient at the last evaluation prior to the start of the preparative regimen / infusion.

Select “Non-transfused (NTD)” if the recipient was without RBC transfusions as supportive care for the disease within a period of 16 weeks prior to the start of the preparative regimen / infusion.

Select “Low-transfusion burden (LTB)” if the recipient had 3-7 RBC transfusions within a period of 16 weeks in at least 2 transfusion episodes with a maximum of 3 RBC transfusions in 8 weeks prior to the start of the preparative regimen / infusion.

Select “High-transfusion burden (HTB)”- if the recipient had ≥8 RBCs transfusions within a period of 16 weeks or ≥4 within 8 weeks prior to the start of the preparative regimen / infusion.

Questions 311: Did the recipient have constitutional symptoms ( > 10% weight loss in six months, night sweats, unexplained fever higher than 37.5°C) in six months before the last evaluation prior to the start of the preparative regimen / infusion?

Report “yes” if constitutional symptoms were present within six months before the last evaluation prior to the preparative regimen / infusion. Constitutional symptoms are often called “B” symptoms and include unexplained fever greater than 38°C (100.4°F), night sweats, or unexplained weight loss in the six months before the last evaluation prior to the start of the preparative regimen / infusion.

Report “no” if constitutional symptoms were not present at this timepoint.

Report “unknown” if it is not possible to determine the presence or absence of constitutional symptoms at this timepoint.

Question 312: Did the recipient have splenomegaly at last evaluation prior to the start of the preparative regimen / infusion?

Indicate if the recipient had splenomegaly at the last evaluation. Splenomegaly is often documented during the physician’s physical assessment of the recipient and represents an abnormal finding. Splenomegaly can also be detected by imaging techniques such as ultrasonography, CT or MRI.

Indicate “yes” if splenomegaly was present at the last evaluation prior to the start of the preparative regimen / infusion and continue with question 313.

Indicate “no” if splenomegaly was not present at the last evaluation and continue with question 316.

Indicate “unknown” if it is not possible to determine the presence or absence of splenomegaly at this timepoint and continue with question 316.

Indicate “not applicable” if the question does not apply to the recipient (e.g. prior splenectomy) and continue with question 316.

Question 313: Specify the method used to measure spleen size

Indicate the method used to measure the spleen size.

If the method selected is “physical assessment,” continue with question 314.

If the method selected is “ultrasound” or “CT / MRI” continue with question 315.

If spleen size is measured using multiple methods, report the most accurate assessment. Ultrasound is the most specific and preferred assessment.

Question 314: Specify the spleen size below the left coastal margin

Indicate the size of the spleen in centimeters, measured below the left coastal margin as assessed by physical exam then continue with question 316.

Question 315: Specify the spleen size in centimeters

Indicate the size of the spleen in centimeters, as assessed by imaging (ultrasound, CT / MRI) then continue with question 316.

Question 316: Did the recipient have hepatomegaly

Indicate if the recipient had hepatomegaly at the last evaluation prior to the start of the preparative regimen / infusion. Hepatomegaly is often documented during the physician’s physical assessment of the recipient and represents an abnormal finding.

Indicate “yes” if hepatomegaly was present at the last evaluation and continue with question 317.

Indicate “no” if hepatomegaly was not present at this timepoint and continue with question 320.

Indicate “unknown” if it is not possible to determine the presence or absence of hepatomegaly at this timepoint and continue with question 320.

Question 317: Specify the method used to measure liver size

Indicate the method used to measure the liver size.

If the method selected is “physical assessment” continue with question 318.

If the method selected is “ultrasound” or “CT / MRI” continue with question 319. If liver size is measured using multiple methods, report the most accurate assessment. Ultrasound is the most specific and preferred assessment.

Question 318: Specify the liver size below the right coastal margin

Indicate the size of the liver in centimeters, measured below the right coastal margin as assessed by physical exam then continue with question 320.

Question 319: Specify the liver size in centimeters

Indicate the size of the liver in centimeters, as assessed by imaging (ultrasound, CT / MRI) then continue with question 320.

Question 320: Date CBC drawn

Report the date of the CBC was drawn at the last evaluation prior to the start of the preparative regimen / infusion and continue with question 321. If multiple CBCs were drawn, report the most recent one prior to the start of the preparative regimen / infusion.

Question 321-322: WBC

Indicate whether the white blood cell (WBC) count was “known” or “unknown” at the last evaluation prior to the start of the preparative regimen infusion / infusion. If “known,” report the laboratory count and unit of measure documented on the laboratory report in question 322. If “unknown,” continue with question 323.

Questions 323-324: Neutrophils

Indicate whether the neutrophil percentage in the blood was “known” or “unknown” at the last evaluation prior to the start of the preparative regimen / infusion. If “known,” report the value documented on the laboratory report in question 324. If “unknown,” continue with question 325.

Question 325-326: Blasts in the blood

Indicate whether the percent blasts in the peripheral blood is “known” or “unknown” at the last evaluation prior to the start of the preparative regimen / infusion.

If “known,” report the laboratory value in question 326. Note, blasts are not typically found in the peripheral blood. If blasts are not noted on the differential, you can still indicate “known” and report “0%” in question 326.

If the percent blasts in blood at the last evaluation prior to the start of the preparative regimen / infusion is not known, report “unknown” and go to question 327.

Question 327-328: Hemoglobin

Indicate whether the hemoglobin was “known” or “unknown” at the last evaluation prior to the start of the preparative regimen / infusion. If “known,” report the laboratory value and unit of measure documented on the laboratory report in question 328. If “unknown,” continue with question 330.

Question 329: Was RBCs transfused ≤ 30 days before the CBC sample date?

Transfusions temporarily increase the red blood cell count. It is important to distinguish between a recipient whose body is creating these cells and a recipient who requires transfusions to support the counts.

Indicate if red blood cells were transfused less than or equal to 30 days prior to the CBC sample date reported in question 320.

Question 330-331: Platelets

Indicate whether the platelet count was “known” or “unknown” at the last evaluation prior to the start of the preparative regimen / infusion. If “known,” report the laboratory count and unit of measure documented on the laboratory report in question 331. If “unknown,” continue with question 333.

Question 332: Were platelets transfused ≤ 7 days before date of test?

Transfusions temporarily increase the platelet count. It is important to distinguish between a recipient whose body is creating the platelets and a recipient who requires transfusions to support the counts.

Indicate if platelets were transfused less than or equal to 7 days prior to the CBC sample date reported in question 320.

Questions 333-334: Blasts in bone marrow:

Indicate whether the percentage of blasts in the bone marrow was “known” or “unknown” at the last evaluation prior to the start of the preparative regimen / infusion. If “known,” report the percentage documented on the pathology report in question 334. If “unknown,” continue with question 335.

Question 335-344: Were tests for driver mutations performed?

Testing for driver mutations may be performed by different methods including next generation sequencing (NGS), polymerase chain reaction (PCR), microarray, and fluorescence in situ hybridization (FISH). If testing was performed by any / all of these methods at the last evaluation prior to the start of the preparative regimen / infusion, report “yes” and report the results for the most recent test(s) in questions 336-344.

If testing for driver mutations were not performed or is unknown, report “no” or “unknown” and continued with question 346.

Question 345: Was documentation submitted to the CIBMTR (e.g. pathology report used for diagnosis)?

Indicate whether documentation was submitted to the CIBMTR (e.g., pathology report). For further instructions on how to attach documents in FormsNet3 SM, refer to the Training Guide.

Question 346: Were cytogenetics tested (karyotyping or FISH)?

Cytogenetics is the study of chromosomes. Cytogenetic assessment involves testing blood or bone marrow for the presence of known chromosomal abnormalities that reflect the recipient’s disease. Testing methods include conventional chromosome analysis (karyotyping) or fluorescence in situ hybridization (FISH). For more information about cytogenetic testing and terminology, see Appendix C, Cytogenetic Assessments.

Indicate if cytogenetic studies were obtained at the last evaluation prior to the preparative regimen / infusion. If cytogenetic studies were obtained, select “yes” and continue with question 347.

If no cytogenetic studies were obtained or it is unknown if chromosome studies were performed, select “no” or “unknown” and continue with question 363.

Question 347: Were cytogenetics tested via FISH?

If FISH studies were performed at the last evaluation prior to the start of the preparative regimen / infusion, report “yes” for question 347 and continue with question 348. If FISH studies were not performed at this time point, report “no” for question 347 and go to question 355. Examples include: no FISH study performed, or FISH sample was inadequate. See Appendix C, Cytogenetic Assessments, for assistance interpreting FISH results.

Report chromosomal microarrays / chromosomal genomic arrays as FISH assessments.

Question 348: Sample source:

Indicate if the sample was from “bone marrow” or from “peripheral blood” and continue with question 348. If multiple sources were used to test FISH, the most preferred sample is the bone marrow.

Question 349: Results of tests:

If FISH assessments identified abnormalities, indicate “abnormalities identified” and continue with question 350.

If FISH assessments were unremarkable, indicate “no abnormalities” identified, continue with question 354.

Questions 350-353: Specify cytogenetic abnormalities (FISH)

Report the International System for Human Cytogenetic Nomenclature (ISCN) compatible string, if applicable, in question 350, then continue with question 351.

Report the number of abnormalities detected by FISH at the last evaluation prior to the preparative regimen / infusion in question 351. After indicating the number of abnormalities in question 352, select all abnormalities detected in question 352.

If a clonal abnormality is detected, but not listed as an option in question 353, select “other abnormality” and specify the abnormality in question 353. If multiple “other abnormalities” were detected, report “see attachment” in question 353 and attach the final report(s) for any other abnormalities detected. For further instructions on how to attach documents in FormsNet3SM, refer to the Training Guide.

Question 354: Was documentation submitted to the CIBMTR?

Indicate whether documentation was submitted to the CIBMTR (e.g., pathology report, FISH report). For further instructions on how to attach documents in FormsNet3SM, refer to the Training Guide.

Question 355: Were cytogenetics tested via karyotyping?

If karyotyping was performed at the last evaluation prior to the preparative regimen / infusion, report “yes” and continue with question 356. If karyotyping was not performed at this time point, indicate “no” and continue with 363. Examples of this include: karyotyping was not performed, or karyotyping sample was inadequate.

Question 356: Sample source:

Indicate if the sample was from “bone marrow” or from “peripheral blood” and continue with question 357. If multiple sources were used to for karyotyping analyses, the most preferred sample is the bone marrow.

Question 357: Results of tests:

If karyotyping assessments identified abnormalities, indicate “abnormalities identified” and continue with question 358.

If karyotyping assessments yielded no evaluable metaphases or there were no abnormalities identified, indicate such and continue with question 362.

Question 358-361: Specify cytogenetic abnormalities (karyotyping)

Report the International System for Human Cytogenetic Nomenclature (ISCN) compatible string, if applicable, in question 358, then continue with question 359.

Report the number of abnormalities detected by karyotyping at the last evaluation prior to the start of the preparative regimen / infusion. After indicating the number of abnormalities in question 359, select all abnormalities detected in question 360.

If a clonal abnormality is detected, but not listed as an option in question 360, select “other abnormality” and specify the abnormality in question 361. If multiple “other abnormalities” were detected, report “see attachment” in question 361 and attach the final report(s) for any other abnormalities detected.

For further instructions on how to attach documents in FormsNet3 SM, refer to the Training Guide.

Question 362: Was documentation submitted to the CIBMTR?

Indicate whether documentation was submitted to the CIBMTR (e.g., karyotype report). For further instructions on how to attach documents in FormsNet3SM, refer to the Training Guide.

Question 363: What was the disease status?

Indicate the disease status of MPN at the last assessment prior to the start of the preparative regimen / infusion. Refer to the MPN Response Criteria section of the Forms Instructions Manual for definitions of each disease response.

If the disease status is “Clinical Improvement (CI)” continue with question 364. If the disease status is “Not Assessed” continue with question 368. For all other disease statuses go to question 367.

Question 364: Was an anemia response achieved?

Specify if an anemia response has been achieved at the last evaluation prior to the preparative regimen / infusion and continue with question 365.

An anemia response is characterized by a ≥20 g/L (or >2.0 g/dL) increase in hemoglobin level (for transfusion-independent recipients) or by becoming transfusion-independent (transfusion-dependent recipients).

Question 365: Was a spleen response achieved?

Specify if a spleen response has been achieved at the last evaluation prior to the preparative regimen / infusion and continue with question 366.

A spleen response is achieved when a baseline splenomegaly that is palpable at 5-10 cm below the left costal margin (LCM) becomes not palpable or baseline splenomegaly that is palpable at >10 cm below the LCM, decreases by ≥50%.

A baseline splenomegaly that is palpable at <5 cm, below the LCM, is not eligible for spleen response.

A spleen response can be documented by a physician or confirmed by MRI / computed tomography showing ≥35% spleen volume reduction.

Question 366: Was a symptom response achieved?

The Myeloproliferative Neoplasm Symptom Assessment Form Total Symptom Score (MPN-SAF TSS) is used to evaluate the recipient’s symptom response. The MPN-SAF TSS is used to provide an accurate assessment of MPN symptom burden. The evaluation tool allows recipients with MPN to report their symptom severity at the worst level. They rate their symptom severity on a scale from zero to ten, zero being absent to ten being the worst imaginable. Adding the scores for all symptoms together will result in the recipient’s MPN-SAF TSS. See Table 1 below for an example of this assessment:

Table 1. Myeloproliferative Neoplasm Symptom Assessment Form Total Symptom Score (MPN-SAF TSS)

Symptom 1 to 10 (0 if absent) ranking – 1 is most favorable and 10 least favorable
Please rate your fatigue (weariness, tiredness) by circling the one number that best describes your WORST level of fatigue during the past 24 hours (No fatigue) 0 1 2 3 4 5 6 7 8 9 10 (Worst imaginable)
Circle the one number that describes how, during the past week how much difficulty you have had with each of the following symptoms.
Filling up quickly when you eat (early satiety) (Absent) 0 1 2 3 4 5 6 7 8 9 10 (Worst imaginable)
Abdominal discomfort (Absent) 0 1 2 3 4 5 6 7 8 9 10 (Worst imaginable)
Inactivity (Absent) 0 1 2 3 4 5 6 7 8 9 10 (Worst imaginable)
Problems with concentration – Compared to prior to my MPD (Absent) 0 1 2 3 4 5 6 7 8 9 10 (Worst imaginable)
Numbness / tingling (in my hands and feet) (Absent) 0 1 2 3 4 5 6 7 8 9 10 (Worst imaginable)
Night sweats (Absent) 0 1 2 3 4 5 6 7 8 9 10 (Worst imaginable)
Itching (pruritus) (Absent) 0 1 2 3 4 5 6 7 8 9 10 (Worst imaginable)
Bone pain (diffuse not joint pain or arthritis) (Absent) 0 1 2 3 4 5 6 7 8 9 10 (Worst imaginable)
Fever (>100 F) (Absent) 0 1 2 3 4 5 6 7 8 9 10 (Worst imaginable)
Unintentional weight loss last 6 months (Absent) 0 1 2 3 4 5 6 7 8 9 10 (Worst imaginable)

A symptom response is achieved when there is a ≥50% reduction in the Myeloproliferative Neoplasm Symptom Assessment Form Total Symptom Score (MPN-SAF TSS).

Specify if a symptom response has been achieved at the last evaluation prior to preparative regimen / infusion and continue with question 367.

Question 367: Date assessed:

Enter the date of the most recent assessment of disease status prior to the start of the preparative regimen / infusion. The date reported should be that of the most disease-specific assessment within the pre-transplant work-up period (approximately 30 days). Clinical and hematologic assessments include pathological evaluation (e.g., bone marrow biopsy), radiographic examination (e.g., X-ray, CT scan, MRI scan, PET scan), and laboratory assessment (e.g., CBC, peripheral blood smear), in addition to clinician evaluation and physical examination. Enter the date the sample was collected for pathological and laboratory evaluations; enter the date the imaging took place for radiographic assessments.

If the exact date is not known, use the process for reporting partial or unknown dates as described in General Instructions, Guidelines for Completing Forms.

Question 368: Specify the cytogenetic response:

Specify the recipient’s cytogenetic response at the last evaluation prior to the start of the preparative regimen / infusion.

If there is eradication of the previous reported abnormality select “Complete response (CR)” and continue with question 369.

If there is a ≥ 50% reduction in abnormal metaphases, select “Partial Remission (PR)” and continue with question 369.

Select “Re-emergence of pre-existing cytogenetic abnormality” if the cytogenetic abnormality was eradicated and reemerged at the last evaluation and continue with question 369.

If cytogenetic response was not tested at the last evaluation select “Not assessed” and continue with question 370.

Select “not applicable” if cytogenetic abnormalities were never identified and continue with question 370.

If the recipient does not meet the criteria for CR or PR, select “None of the above” and continue with question 370 (e.g. if a new cytogenetic abnormality is identified but there is also eradication of a previous abnormality).

Example: A recipient had 10 abnormal metaphases (out of 20) at diagnosis. At the last evaluation prior to the start of the preparative regimen, they had 2 abnormal metaphases (out of 20). As this is a ≥50% reduction in abnormal metaphases, “Partial Remission (PR)” should be reported.

Question 369: Date assessed:

Report the date the cytogenetic response was established. Enter the date the sample was collected for pathologic evaluation (e.g., bone marrow biopsy) or blood/serum assessment (e.g., CBC, peripheral blood smear).

Question 370: Specify the molecular response:

Specify the recipient’s molecular response at the last evaluation prior to the start of the preparative regimen / infusion, based on the four drive mutations (JAK2, CALR, MPL, and CSF3R) listed in questions 279 – 287.

If there is eradication of the previously reported driver mutation (JAK2, CALR, MPL, and/or CSF3R), select “Complete response (CR)” and continue with question 371.

If there is a 50% decrease in allele burden of the driver mutation (JAK2, CALR, MPL, and/or CSF3R), select “Partial Remission (PR)” and continue with question 371.

Example: A recipient was found to have a molecular mutation identified (JAK2, CALR, MPL, and/or CSF3R) in 80% of cells examined at diagnosis. At their last evaluation prior to transplant, the molecular mutation was only identified in 40% of cells examined. The number of cells with the molecular mutation identified decreased from 80% to 40%, which is a 50% reduction. In this case, “Partial Remission” should be reported as their molecular response.

Select “Re-emergence of pre-existing molecular abnormality” if the molecular abnormality (JAK2, CALR, MPL, and/or CSF3R) was eradicated and reemerged at the last evaluation and continue with question 371.

Select “not applicable” if JAK2, CALR, MPL, and CSF3R were never identified and go to first name.

If molecular response was not tested at the last evaluation select “Not assessed” and go to first name. If the recipient does not meet the criteria for CR or PR select “None of the above” and go to first name.

Question 371: Date assessed:

Report the date the molecular response was established. Enter the date the sample was collected for pathologic evaluation (e.g., bone marrow biopsy) or blood/serum assessment (e.g., CBC, peripheral blood smear).

Last modified: Oct 23, 2020

Need more help with this?
Don’t hesitate to contact us here.

Was this helpful?

Yes No
You indicated this topic was not helpful to you ...
Could you please leave a comment telling us why? Thank you!
Thanks for your feedback.