Question 253: Specify the recipient’s current hematologic status:

Indicate the recipient’s current hematologic status at the time of evaluation for this reporting period. See the Amyloidosis Response Criteria section for disease status definitions.

The percentage of plasma cells in the bone marrow aspirate may also be identified on a flow cytometry report. A flow cytometry report may be used to confirm CR (e.g., < 5% plasma cells in the bone marrow) as long as the method was high sensitivity or next generation flow.

If the disease response prior to transplant is unknown, select “unknown” and continue with question 256.

Questions 254-255: Date assessed:

Indicate if the assessment date that reflects the recipient’s current hematologic status was “known” or “unknown.” If “known,” continue with question 255 and enter the date of the most recent disease evaluation. Report the date the blood/urine was collected for the laboratory evaluations (e.g., SPEP/UPEP, serum/urine immunofixation) or report the date the bone marrow was collected for pathological evaluation. A PET scan may be used if a PET scan was previously obtained and only in limited circumstances (e.g., plasmacytomas, lytic lesions). If “unknown,” continue with question 256.

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

Question 256: Specify the recipient’s current cardiac response:

Indicate the recipient’s current cardiac status at the time of evaluation for this reporting period. See the Amyloidosis Response Criteria section for disease status definitions.

If the recipient’s cardiac status was not assessed during the reporting period, select “not assessed” and continue with question 290. If the recipient never had evidence of cardiac involvement in their disease, select “not applicable” and continue with question 290.

Questions 257-258: Date assessed:

Indicate if the date of current cardiac assessment is “known” or “unknown.” If the date of assessment for cardiac status is known, report the date in question 258. If the date is unknown, select “unknown” and continue with question 259.

Question 259: Was the left ventricular ejection fraction measured?

The left ventricular ejection fraction (LVEF) is a percentage that represents the volume of blood pumped from the left ventricle into the aorta (also known as stroke volume) compared to the volume of blood in the ventricle just prior to the heart contraction (also known as end diastolic volume). Indicate if the left ventricular ejection fraction (LVEF) was measured. If “yes,” continue with question 260. If “no,” continue with question 262.

Question 260: Specify the left ventricular ejection fraction:

Indicate the left ventricular ejection fraction at the time of evaluation for this reporting period. Most imaging reports will report the LVEF. If the LVEF is not explicitly documented it should be determined by dividing the stroke volume (SV, the volume of blood pumped into the aorta from the left ventricle) by the end diastolic volume (EDV, the volume of blood in the left ventricle just prior to contraction) of the left ventricle. For example, if the stroke volume was 75 ml and the end diastolic volume was 150ml, the ejection fraction would be 50%.

Question 261 : Specify the method used to determine the left ventricular ejection fraction:

Indicate the method used to determine the LVEF value.

Question 262: Was diastolic dysfunction present?

Diastole is the period in which chambers of the heart fill with blood. Diastolic dysfunction may be characterized by the difficulty of the ventricles to expand and contract appropriately due to stiffening of the heart walls by amyloid deposits. Indicate if diastolic dysfunction was present. Specify “yes,” “no,” or “unknown,” and continue with question 263.

Questions 263-264: Specify the intraventricular septal wall thickness measured by echocardiogram:

The heart is divided into the right and left sides by the septum. The area between the left and right ventricles is the intraventricular septum. Indicate if the intraventricular septal thickness is “known” or “unknown.” If “known,” based on evaluation by echocardiogram, indicate the thickness of the intraventricular septal wall in question 264. If “unknown,” or not measured by echocardiogram, continue with question 265.

Questions 265-266: Specify left ventricular (LV) strain percentage:

A strain pattern, as determined by electrocardiography, is a well-recognized marker of hypertrophy of the left ventricular (LVH) and is characterized by ST depression and T wave inversion on a resting ECG / EKG. The LV strain percentage is typically a negative percentage. The normal range for the LV global longitudinal strain (LV GLS) is -15.9% to -22.1%. Indicate if the left ventricular strain percentage is “known” or “unknown.” If “known,” based on evaluation by electrocardiogram, indicate the strain percentage in question 266. If “unknown,” or not measured by electrocardiogram, continue with question 267.

Questions 267-268: Were any serum cardiac biomarkers assessed?

Assessment of cardiac biomarkers helps determine if injury to cardiac tissue has occurred. Cardiac biomarkers include brain natriuretic peptide (BNP), N-terminal prohormone brain natriuretic peptide (NT-proBNP), troponin I, troponin T, and high-sensitivity troponin T. Indicate if serum cardiac biomarkers were assessed at the last evaluation. If “yes,” report the date assessed in question 268 and continue with question 269. If “no” or “unknown,” continue with question 284.

Questions 269-270: Brain natriuretic peptide (BNP):

Indicate if the BNP was assessed at the last evaluation. If “yes,” report the value (in pg/mL) in question 270 and continue with question 271. If “no,” continue with question 272.

Question 271: Upper limit of normal for BNP:

Indicate the upper limit of normal for BNP (in pg/mL) found on the laboratory report.

Questions 272-273: N-terminal prohormone brain natriuretic peptide (NT-proBNP):

Indicate if the NT-proBNP was assessed at the late evaluation. If “yes,” report the value (in pg/mL) in question 273 and continue with question 274. If “no,” continue with question 275.

Question 274: Upper limit of normal for NT-proBNP:

Indicate the upper limit of normal (in pg/mL) for NT-proBNP found on the laboratory report.

Questions 275-276: Troponin I:

Indicate if the Troponin I was assessed at the last evaluation. If “yes,” report the value (in µg/L) in question 276 and continue with question 277. If “no,” continue with question 278.

Question 277: Upper limit of normal for troponin I:

Indicate the upper limit of normal (in µg/L) for Troponin I found on the laboratory report.

Questions 278-279: Troponin T:

Indicate if the Troponin T was assessed at the last evaluation. If “yes,” report the value (in µg/L) in question 279 and continue with question 280. If “no,” continue with question 281.

Question 280: Upper limit of normal for Troponin T:

Indicate the upper limit of normal (in µg/L) for Troponin T found on the laboratory report.

Questions 281-282: High-sensitivity troponin T:

Indicate if the high-sensitivity troponin T was assessed at the last evaluation. If “yes,” report the value (in ng/L) in question 282 and continue with question 283. If “no,” continue with question 284.

Question 283: Upper limit of normal for high-sensitivity troponin T:

Indicate the upper limit of normal (in ng/L) for high-sensitivity troponin T found on the laboratory report.

Questions 284-285: Was a 6-minute walk test performed?

A 6-minute walk test is used to assess total distance walked within 6 minutes to determine aerobic capacity and endurance. Indicate if a 6-minute walk test was performed at the last evaluation. If “yes,” report the total distance walked and specify the unit of measure in question 285. If “no,” continue with question 286.

Question 286: Specify the recipient’s New York Heart Association functional classification of heart failure: (Symptoms may include dyspnea, chest pain, fatigue, and palpitations; activity level should be assessed with consideration for patient’s age group)

Indicate the recipient’s New York Heart Association functional classification at the last evaluation using the following guidelines:

  • Class I – Able to perform ordinary activities without symptoms; no limitation of physical activity
  • Class II – Ordinary physical activity produces symptoms; slight limitation of physical activity
  • Class III – Less-than-ordinary physical activity produces symptoms; moderate limitation of physical activity
  • Class IV – Symptoms present even at rest; severe limitation of physical activity

If the recipient’s NYHA functional classification it not known, select “unknown,” and continue with question 287.

Question 287: Recipient blood pressure:

Indicate if the recipient’s blood pressure was assessed at the last evaluation. If “known,” continue with question 288. If “unknown,” continue with question 290.

Questions 288-289: Recipient blood pressure results:

Report the recipient’s blood pressure at the last evaluation in question 288 and indicate in which body position the measurement was taken in question 289.

Question 290: Specify the recipient’s current renal response:

Indicate the recipient’s best renal response to HCT to date. See the Amyloidosis Response Criteria section for disease status definitions.

If the recipient’s renal status was not assessed during the reporting period, select “not assessed” and continue with question 293.If the recipient never had evidence of renal involvement in their disease, select “not applicable” and continue with question 293.

Questions 291-292: Date assessed:

Indicate if the date the current renal response to transplant was assessed is “known,” or “unknown.” If the date of current renal response is known, report the date in question 292. If the date is unknown, select “unknown” and continue with question 293.

Question 293: Specify the recipient’s current hepatic response:

Indicate the recipient’s best hepatic response to HCT to date. See the Amyloidosis Response Criteria section for disease status definitions.

If the recipient’s hepatic status was not assessed during the reporting period, select “not assessed” and continue with question 300. If the recipient never had evidence of hepatic involvement in their disease, select “not applicable” and continue with question 300.

Questions 294-295: Date assessed:

Indicate if the date the current hepatic response was assessed is “known,” or “unknown.” If the date of current hepatic response is known, report the date in question 295. If the date is unknown, select “unknown” and continue with question 296.

Question 296: Was hepatomegaly present on radiographic imaging (liver span > 15 cm) or on examination (liver edge palpable > 3 cm below right costal margin)?

At the last evaluation, indicate if the liver spanned more than 15 cm (by radiographic imaging) or the edge of the liver was palpable more than 3 cm below the right costal margin (by physical examination). Indicate “yes” if hepatomegaly was present at the last evaluation. Indicate “no” if hepatomegaly was not present at the last evaluation. Indicate “unknown” if it was not possible to determine the presence or absence of hepatomegaly at the last evaluation.

Questions 297-298: Specify the level of serum alkaline phosphatase:

Indicate whether the alkaline phosphatase (ALP) level at the time of evaluation for this reporting period is “known” or “unknown.” If “known,” report the laboratory count and unit of measure documented on the laboratory report in question 298 and continue with question 299. If “unknown,” continue with question 300.

Question 299: Upper limit of normal for alkaline phosphatase:

Report the upper limit of normal for ALP found on the laboratory report.

Question 300: Was there clinical improvement in GI involvement since the date of last report?

Indicate if there was clinical improvement of GI involvement to date. Judgment is required by a clinician to determine if there is evidence of improvement. If “yes” or “no,” continue with question 301. If “unknown” or “not applicable,” continue with question 303. Report “not applicable” if the recipient never had evidence of GI involvement in their disease and continue with question 300.

Questions 301-302: Date assessed:

Indicate if the date the GI involvement was assessed is “known,” “unknown,” or “previously reported.” If the date the GI response was assessed is “known,” report the date in question 302. If the date is unknown, select “unknown” and continue with question 303. If the best response to transplant was already reported in a previous reporting period, select “previously reported” and continue with question 303.

Question 303: Specify the recipient’s current peripheral nervous system response:

Indicate the recipient’s best peripheral nervous system response to HCT to date. See the Amyloidosis Response Criteria section for disease status definitions.

If the recipient’s peripheral neuropathy was not assessed during the reporting period, select “not assessed” and continue with question 306. If the recipient never had evidence of disease-related peripheral neuropathy, select “not applicable” and continue with question 306.

Questions 304-305: Date assessed:

Indicate if the date the current peripheral neuropathy response to transplant was assessed is “known,” or “unknown.” If the date of current peripheral neuropathy response is “known,” report the date in question 305. If the date is unknown, select “unknown” and continue with question 306.

Question 306: Did the recipient display any other clinical organ involvement?

Indicate if any other system was assessed for response to HCT. If the recipient had other site involvement reported in questions 116-118 of the Pre-HCT Plasma Cell Disorder form (Form 2016) and that site was assessed, the response to HCT must be reported here, even if there was no response. If any other system was assessed, report “yes” and continue with question 307. If no other systems were assessed at the time of best response, report “no” and continue with question 312.

Questions 307-308: Specify the evidence of other organ involvement (check all that apply):

For each option, indicate if there was evidence of other organ involvement at the last evaluation. Check all that apply. If there was other organ involvement not listed in this section, select “other organ involvement” in question 307 and specify the other organ in question 308.

Examples may include:

  • Arthropathy is a disease of the joints. An example of a common arthropathy in patients with amyloidosis is carpal tunnel-like symptoms.
  • Amyloid deposits may be found in the lung, impairing their function. Examples of lung involvement may be alveolar-septal disease, nodular disease, intra- and extra-thoracic adenopathy, pleural disease, and diaphragm deposition.5
  • Soft tissue involvement, other than those already listed, may include glandular involvement (such as submandibular glands).
  • Any additional organ involvement, other than those already listed, may be reported in this section as “other organ involvement.” The other organ involved will then be specified in question 308.

5 Berk JL, O’Regan A, Skinner M. Pulmonary and tracheobronchial amyloidosis. Semin Respir Crit Care Med. 2002;23(2):155-65.

Question 309: Specify the current status of this system:

Indicate if the site’s/system’s current status to transplant was “improved response,” “progression,” or “no response / stable disease.”

Questions 310-311: Date assessed:

Indicate if the date the other site’s/system’s current status was assessed is “known,” or “unknown.” If the other site’s/system’s response is “known,” report the date in question 311. If the date is unknown, select “unknown” and continue with question 312.

Last modified: Mar 27, 2020

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