Pulmonary Function

Questions 249 – 252: Did the recipient develop non-infectious interstitial pneumonitis (IPn or ARDS) / idiopathic pneumonia syndrome (IPS)? (Report infectious pneumonia in infection section)

IPn refers to inflammation of the alveolar walls. Acute respiratory distress syndrome (ARDS) typically refers to fluid build-up within the alveoli. In either case, gas exchange is impaired resulting in oxygen deprivation. Both conditions can result from infectious or non-infectious causes.

Idiopathic pneumonia syndrome (IPS) refers to all non-infectious lung injuries that occur early after HCT (within 100-120 days) including: peri-engraftment respiratory distress syndrome (PERDS), interstitial pneumonitis without a pathogen, radiation / drug-induced lung injury, or transfusion-associated lung injury (TRALI).

Report any non-infectious pulmonary abnormalities diagnosed since the date of the last report. See below for a description of reportable abnormalities as well as common methods of assessment.

Indicate Yes or No if the recipient developed non-infectious interstitial pneumonitis (IPn or ARDS) / idiopathic pneumonia syndrome (IPS) in the reporting period. If Yes, report the diagnosis date and the diagnostic method(s), other than radiographic studies. Select all that apply.

If the diagnosis was determined at an outside center and no documentation of a clinical, pathological, or laboratory assessment is available, the dictated date of diagnosis within a physician note may be reported.

For more information regarding reporting partial or unknown dates, see General Instructions, General Guidelines for Completing Forms.

Documentation of diagnostic tests may be attached to the form. For further instructions on how to attach documents in FormsNet3, refer to the training guide.

Diagnostic Methods:

  • Bronchoalveolar lavage (BAL): a procedure in which a bronchoscope is guided into the lower respiratory system. Fluid is emitted from the bronchoscope and then collected for further examination.
  • Transbronchial biopsy: a procedure in which forceps on the end of the bronchoscope are used to collect lung tissue samples for further examination.
  • Open / thorascopic lung biopsy: An open lung biopsy is a procedure in which an incision is made between the ribs to collect a sample of lung tissue for further examination. A thorascopic lung biopsy is a procedure in which an incision is made to the chest and an endoscope is used to collect samples of lung tissue.
  • Autopsy: a post-mortem procedure used to determine the cause of death and to evaluate other disease present at the time of death.

Questions 253 – 258: Specify other non-infectious pulmonary abnormality developed (e.g. bronchiolitis obliterans, COP / BOOP, diffuse alveolar hemorrhage)

Indicate if the recipient developed any other non-infectious pulmonary abnormality.

  • Bronchiolitis obliterans (BO): an airway obstruction as a result of inflammation of the bronchioles. This complication typically occurs late after HCT. It is often a manifestation of chronic GVHD. If bronchiolitis obliterans is a result of chronic GVHD, confirm that bronchiolitis obliterans was also reported in the chronic GVHD section of this form.
  • Cryptogenic organizing pneumonia (COP) / Bronchiolitis obliterans with organizing pneumonia (BOOP): an idiopathic form of pneumonia which affects different parts of the lungs including the bronchioles and alveoli. This complication typically occurs late after HCT.
  • Diffuse alveolar hemorrhage (DAH): bleeding into the alveolar space typically resulting from an injury to the pulmonary blood vessels.
  • Other non-infectious pulmonary abnormality: any other non-infectious pulmonary abnormalities not already captured in the above categories. Do not report pleural effusions here.

If the recipient did not develop any other non-infectious pulmonary abnormality, select None.

If the recipient developed a non-infectious pulmonary abnormality, report the diagnosis date and specify the diagnostic method, other than radiographic studies. Select all that apply.

If the diagnosis was determined at an outside center and no documentation of a clinical, pathological, or laboratory assessment is available, the dictated date of diagnosis within a physician note may be reported.

For more information regarding reporting partial or unknown dates, see General Instructions, General Guidelines for Completing Forms.

Documentation of diagnostic tests may be attached to the form. For further instructions on how to attach documents in FormsNet3, refer to the training guide.

Question 259 – 260: Did the recipient receive endotracheal intubation or mechanical ventilation?

Endotracheal intubation or mechanical ventilation may be used for respiratory failure or for airway protection from severe mucositis.

Invasive positive pressure ventilation is delivered via an endotracheal tube. Do not include non-invasive positive pressure ventilation that is delivered through an alternate interface (e.g., facemask).

Indicate whether the recipient received endotracheal intubation or mechanical ventilation (invasive positive pressure ventilation) post-HCT. If Yes, report the date when endotracheal intubation or mechanical ventilation was started. If the recipient was intubated multiple times within the reporting period, report the first date of intubation. Report No if the patient received endotracheal intubation or mechanical ventilation during surgery.

For more information regarding reporting partial or unknown dates, see General Instructions, General Guidelines for Completing Forms.

Question 261 – 262: Was the recipient successfully extubated?

Indicate if the recipient was successfully extubated during the reporting period. If Yes, report the date of extubation. If the recipient was extubated multiple times within the reporting period, please report the last date of extubation.

For more information regarding reporting partial or unknown dates, see General Instructions, General Guidelines for Completing Forms.

Liver Toxicity Prophylaxis and Function

Questions 263 – 264: Specify therapy used to prevent liver toxicity (check all that apply)

Liver toxicities in transplant patients may be related to drugs / treatments, infection, GVHD, iron overload, cirrhosis, or sinusoidal obstructive syndrome (SOS) / veno-occlusive disease (VOD). Agents such as ursodiol may be given as prophylaxis against one or more of these transplant-related liver injuries. Agents given to prevent liver toxicity will generally be started prior to or during the conditioning regimen and may be continued well after transplant.

Select all therapy the recipient received intended to prevent liver toxicity during the reporting period, including therapy given during the preparative regimen. Report only agents given to prevent liver toxicities, not those given to treat a diagnosed liver injury or toxicity.

Question 265: Did the recipient develop non-infectious liver toxicity (excluding GVHD)?

Indicate Yes or No if the recipient developed a non-infectious liver toxicity during the reporting period. Include toxicities which developed between the start of the preparative regimen and the date of last contact (question 1) when completing the 100-day follow-up form. Do not report liver complications due to GVHD in this section.

Question 266 – 268: Specify the etiology of the non-infectious liver toxicity etiology

Report the etiology of the non-infectious liver toxicity that the recipient developed in the reporting period and specify the diagnosis date.

For more information regarding reporting partial or unknown dates, see General Instructions, General Guidelines for Completing Forms.

  • Veno-occlusive disease (VOD) / sinusoidal obstruction syndrome (SOS): occurs following injury to the hepatic venous endothelium, resulting in hepatic venous outflow obstruction due to occlusion of the hepatic venules and sinusoids. This typically results in a distinctive triad of clinical signs including hepatomegaly with right upper quadrant tenderness, third space fluid retention (e.g., ascites), and jaundice with a cholestatic picture. For more information on VOD / SOS including diagnostic criteria, refer to the VOD / SOS section of the Forms Instructions Manual.
  • Cirrhosis: Cirrhosis is a degenerative disease in which fibrous tissue forms and the lobes become filled with fat. Cirrhosis may be diagnosed using a liver biopsy, but clinical symptoms (enlarged liver), blood tests, laparoscopy, or radiology imaging are often used to determine the diagnosis of cirrhosis when a liver biopsy is not necessary.
  • Other etiology: Liver toxicity other than VOD / SOS or cirrhosis. Do not include hepatic infections or GVHD.
  • Unknown etiology: If there is a liver toxicity; however, there is no information about the etiology of the non-infectious liver toxicity. This option should be used sparingly and only when no judgment can be made about the etiology in the reporting period.

Thrombotic Microangiopathy (TMA)

Thrombotic microangiopathy (TMA) is a multifactorial condition where intravascular platelet activation, formation of thrombi, and microangiopathic hemolytic anemia occur due to generalized endothelial dysfunction. Organ injury, specifically the kidney, may occur as a result of these processes.1 Characteristics of thrombotic microangiopathy includes microangiopathic hemolysis, thrombocytopenia (< 50 ×109/L), neurological changes, and pulmonary dysfunction: Other laboratory features include:

  • LDH greater than the center-specific upper limit of normal
  • Serum creatinine > 2 mg/dL or >50% rise over baseline
  • Bilirubin greater than twice the center-specific upper limit of normal

1 Jodele, S., M. Davies, and B.L. Laskin. “Diagnostic and Risk Criteria for HSCT-associated Thrombotic Microangiopathy: A Study in Children and Young Adults.” Blood 124.4 (2014): 645-53. Web.

Questions 269 – 270: Did the recipient develop post-infusion thrombotic microangiopathy (TMA) or similar syndrome? (includes microangiopathy, thrombotic thrombocytopenia purpura (TTP), hemolytic uremic syndrome (HUS))

Indicate Yes or No if the recipient developed post-infusion TMA or a similar syndrome since the date of last report. If Yes, report the diagnosis date.

For more information regarding reporting partial or unknown dates, see General Instructions, General Guidelines for Completing Forms.

  • Microangiopathy: Disease of the capillaries where the capillaries bleed and slow the flow of blood due to thickening and weakening of capillary walls.
  • Thrombotic thrombocytopenia (TTP): Blood disorder where blood clots form in the small blood vessels of the body.
  • Hemolytic uremic syndrome (HUS): Abnormal destruction of red blood cells which block the kidneys resulting in kidney failure. May be caused by Escherichia coli, other infections, and medications.

If the recipient did not develop post-infusion TMA or a similar syndrome, report No.

Question 271: Specify signs and symptoms (check all that apply)

Report all signs or symptoms the recipient experienced due to post-infusion TMA or a similar syndrome during the reporting period. Select all that apply.

Questions 272 – 276: Was TMA evaluated by biopsy?

Indicate whether TMA was evaluated by a biopsy.

If Yes, report if the results as Positive, Suggestive, Negative, Inconclusive / equivocal, or Not done. If Other site is reported, specify the biopsy site.

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

Questions 277 – 278: Was therapy given for TMA?

Specify any therapy given in the reporting period for TMA. Report only agents given to treat a diagnosis of TMA. If no therapy was given, select None.

Questions 279 – 280: Did the TMA resolve? (Normalization of renal function, LDH, and resolution or improvement in renal and / or neurologic dysfunction)

Indicate whether TMA resolved. If Yes, report the first date the recipient met the following criteria:

  • Normalization of renal function (per institutional guidelines);
  • Normalization of LDH (per institutional guidelines);
  • Resolution / improvement of renal and neurologic dysfunction.

Other Organ Impairment / Disorder

The intent of this section is to identify serious conditions or impairments occurring after transplant. For the purposes of this manual, the term “clinically significant” refers to conditions requiring treatment or intervention. Additional guidelines for commonly reported organ impairments and disorders are included below. Do not report complications that are expected for most transplant recipients and do not require treatment (i.e., minor complications resolving without intervention).

Report any other clinically significant organ impairment or disorder the recipient developed during the reporting period. If this form is being completed for the 100-day reporting period, include any clinically significant impairments or disorders diagnosed between the start of the preparative regimen and the date of contact (question 1).

Do not report any impairments / disorders which have persisted since the last report. If this form is being completed for the 100-day reporting period, do no report conditions which have persisted since before the start of the preparative regimen.

Questions 281 – 305: Specify impairment / disorder

Indicate if any of the organ impairments or disorders listed were diagnosed during the reporting period. If the recipient developed an impairment during the reporting period, select the impairment / disorder, enter the date of diagnosis, and answer any additional questions pertaining to the impairment / disorder. If the diagnosis was determined at an outside center and no documentation of a clinical, pathological, or laboratory assessment is available, the dictated date of diagnosis within a physician note may be reported.

Do not report organ impairments or disorders diagnosed prior to the start of the preparative regimen / infusion or in a prior reporting period.

For more information regarding reporting partial or unknown dates, see General Instructions, General Guidelines for Completing Forms.

Renal

  • Acute renal failure requiring dialysis: report whether dialysis was ordered or recommended for renal failure. Also report whether the recipient received the treatment. Symptoms of renal failure include dehydration, nausea, blood in the urine, and / or swelling of extremities.
  • Chronic kidney disease / renal impairment: report whether there was chronic kidney disease or renal impairment (persistent decrease in glomerular filtration to < 60 mL/min.1.73m2). Also report whether the recipient received treatment. If this is selected, also indicated if the recipient was placed on dialysis.

Cardiac

  • Arrhythmia: report whether the recipient developed an arrhythmia, including atrial fibrillation or flutter, sick sinus syndrome, and ventricular arrhythmia. If selected, specify the type of arrhythmia.
  • Cardiomyopathy: a disease of the heart muscle that makes it more difficult for the heart to pump blood to the rest of the body.
  • Congestive heart failure (CHF): inability of the heart to supply oxygenated blood to meet the body’s needs. Ejection fraction < 40%. If selected, report the ejection fraction and specify if the recipient was Symptomatic or Asymptomatic.
  • Coronary artery disease: damage or disease in the major blood vessels of the heart. Also called CAD, atherosclerotic heart disease.
  • Unstable angina: sometimes called acute coronary syndrome and results in unexpected chest pain due to reduced blood flow and oxygen to the heart.
  • Myocardial infarction (MI): an obstruction in the coronary artery resulting in damage / necrosis to the cardiac muscle.
  • Hypertension (HTN) requiring therapy: report whether the recipient was still receiving therapy for hypertension on the contact date for the reporting period.
  • Pericarditis: swelling and irritation of the pericardium.
  • Heart valve disease: the presence of one or more of the following:
    • Moderate or severe degree of valve stenosis or insufficiency as determined by echo, whether the valve is mitral, aortic, tricuspid or pulmonary
    • Prosthetic mitral or aortic valve
    • Symptomatic mitral valve prolapse

Vascular

  • Deep vein thrombosis (DVT): development of a blood clot in a deep vein. Specify if the DVT was catheter (central line) related in question 291. This information is typically documented within the results of the ultrasound.
  • Pulmonary embolism (PE): development of a blood clot in the arteries of the lung. Specify if the PE was catheter (central line) related in question 292. This information is typically documented within the results of the ultrasound.
  • Hyperlipidemia (high total cholesterol, low HDL cholesterol, high LDL cholesterol, and/or high triglyceride levels): high levels of lipids (fat particles) in the blood. Hyperlipidemia is typically diagnosed by a lipid panel. If this is reported, report the diagnosis date as the date when the lipid panel was drawn. Additionally, specify which lipids were assessed and provide the results (fasting results are preferred). Check all apply.

Neurological

  • CNS hemorrhage: bleeding within the central nervous system.
  • Encephalopathy: damage or disease of the brain. Symptoms of encephalopathy include memory loss, personality changes, and declining ability to concentrate and reason.
  • Neuropathy: nerve damage, usually in hands and feet, which causes pain, weakness, and numbness.
  • Seizures: sudden, involuntary muscle contractions due to the hyperexcitation of neurons.
  • Stroke / transient ischemic attack: loss of brain function due to a disturbance in the blood supply to the brain. If the recipient experienced different kinds of strokes in the reporting period, report a separate instance for each type of stroke.

Endocrine

  • Diabetes / hyperglycemia requiring chronic treatment: high blood glucose levels. Diabetes / hyperglycemia should only be reported if insulin and / or oral medication is required for treatment. Diabetes / hyperglycemia controlled through diet and exercise should not be reported. Indicate if the recipient was still receiving treatment for diabetes / hyperglycemia on the contact date for the reporting period. Only select this option if the recipient developed diabetes / hyperglycemia requiring treatment post-infusion. Do not report this disorder if the recipient had a diagnosis and was on treatment prior to infusion.
  • Growth hormone deficiency / short stature: a condition in which the body does not produce enough growth hormone / a reduced overall rate of growth. Indicate if the recipient received therapy for the growth hormone deficiency / short stature.
  • Hypothyroidism requiring replacement therapy: decreased activity of the thyroid gland. Diagnosis of hypothyroidism includes high levels of thyroid-stimulating hormone (TSH). Symptoms of hypothyroidism include fatigue, depression, weakness, weight gain, musculoskeletal pain, decreased taste, hoarseness, and / or puffy face.
  • Pancreatitis: inflammation of the pancreas.

Genitourinary

  • Gonadal dysfunction requiring hormone replacement (testosterone or estrogen): Females may experience early symptoms of menopause including amenorrhea. Males may experience decreased spermatogenesis. Low levels of follicle stimulating hormone (FSH), luteinizing hormone (LH), and / or testosterone may require hormone replacement therapy.
  • Hemorrhagic cystitis / hematuria requiring medical intervention (catheterization of bladder, extra transfusions, urology consult): characterized by bleeding and inflammation of the bladder wall. Hemorrhagic cystitis may result from systemic chemotherapy or radiation therapy and / or some viral infections (e.g., BK virus). Report cases with macroscopic (visible to the naked eye) or gross hematuria (WHO Grade III and IV hemorrhagic cystitis). If the etiology is infectious, also report in the Infection section. Examples of medical intervention include catheterization of bladder, extra transfusions, or a urology consult.

Musculoskeletal

  • Avascular necrosis: localized tissue death due to inadequate oxygen to the cells. Also known as coagulation necrosis or ischemic necrosis.
  • Osteonecrosis of the jaw: bones of the jaw weaken and die due to potent antiresorptive medications such as bisphosphonates or RANKL inhibitors, infection, steroid use, and treatment of cancer, including radiation.
  • Osteoporosis: bones become weak and brittle due to losing bone mass faster than it is created from aging.
  • Osteoporotic fracture: fractures due to low bone mineral density.

Psychiatric

  • Depression requiring therapy: mood disorder resulting in persistent feeling of sadness and loss of interest. Common treatments include antidepressant, anxiolytic, and antipsychotic medications. Common names include Amitriptyline, Bupropion (Wellbutrin), Buspirone, and Abilify.
  • Anxiety requiring therapy: disorder characterized by feelings of worry, anxiety, or fear which are strong enough to interfere with daily activities. Common medications include Duloxetine (Cymbalta), Diazepam (Valium), Buspirone, Pregabalin (Lyrica).
  • Post-traumatic stress disorder (PTSD) requiring therapy: condition triggered by seeing or experiencing a traumatic event.

Other

  • Cataracts: loss of transparency in the lens of the eye.
  • Iron overload requiring therapy: condition characterized by having too much iron in the body. Therapy includes phlebotomy and iron chelation. Indicate which therapy is required. Check all apply.

Iron overload cannot be answered on the day 100 form. Iron overload questions will be answered for all subsequent reporting periods.

  • Mucositits requiring therapy: inflammation and ulceration of mucous membranes that line the digestive tract, usually due to chemotherapy and radiotherapy. Specify the grade as 0 (none), I (mild) – oral soreness, erythema, II (moderate) – oral erythema, ulcers, solid diet tolerated, III (severe) – oral ulcers, liquid diet only, or IV (life-threatening – oral ulcers, oral alimentation impossible in question 303. Do not report mucositis which did not require treatment or intervention during the reporting period.

Mucositis can only be answered on the day 100 form. Mucositis questions will be skipped for all subsequent reporting periods.

  • Other impairment or disorder: use this category to report any clinically significant impairment(s) / disorder(s) not listed on the form. Specify the other impairment / disorder.

Do not report complications that have been reported elsewhere on the form.

  • None: If there are no other organ impairments / disorders, select this option.

Questions 306 – 310: Has the recipient received a solid organ transplant since the date of last report?

Indicate whether the recipient received a solid organ transplant since the date of the last report. If Yes, specify the organ transplanted. If Other organ is reported, specify organ. Additionally, report the date of the solid organ transplant and specify the solid organ donor type.

For more information regarding reporting partial or unknown dates, see General Instructions, General Guidelines for Completing Forms.

If the recipient did not receive a solid organ transplant during the reporting period, report No.

Solid organ transplant questions cannot be answered on the day 100 forms. These questions will be answered for all subsequent reporting periods.

Section Updates:

Question Number Date of Change Add/Remove/Modify Description Reasoning (If applicable)
. . . . .
Last modified: Sep 23, 2022

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