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Acute Myelogenous Leukemia Pre-HSCT Data

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Instructions for Acute Myelogenous Leukemia Pre-HSCT Data (Form 2010) This section of the CIBMTR Forms Instruction Manual is intended to be a resource for completing the AML Pre-HSCT Data Form.
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Instructions for Acute Myelogenous Leukemia Pre-HSCT Data (Form 2010) This section of the CIBMTR Forms Instruction Manual is intended to be a resource for completing the AML Pre-HSCT Data Form. comments regarding the content of the CIBMTR Forms Instruction Manual to: Comments will be considered for future manual updates and revisions. For questions that require an immediate response, please contact your transplant center s CIBMTR liaison. TABLE OF CONTENTS Key Fields... 2 Disease Assessment at Diagnosis... 2 Laboratory Studies at Diagnosis Pre-HSCT Treatment for Acute Myelogenous Leukemia Laboratory Studies Prior to the Start of the Preparative Regimen Disease Status at the Last Assessment Prior to the Preparative Regimen Acute Myelogenous Leukemia Pre-HSCT Data AML is a cancer of the blood and bone marrow. Healthy bone marrow produces immature cells (normal blasts) that then develop into white blood cells. White blood cells (neutrophils) help fight infection. In AML, the blasts do not mature normally into healthy white blood cells. Instead, the abnormal leukemic blasts reproduce rapidly, crowding out healthy white blood cells, red blood cells, and platelets that the body needs. Symptoms of AML infections, fatigue, unusual bleeding result from the lower-than-normal levels of these cells. The Acute Myelogenous Leukemia Pre-HSCT Data Form is one of the Comprehensive Report Forms. This form captures AML-specific pre-hsct data such as: the recipient s hematologic and cytogenetic findings at the time of diagnosis and prior to the start of the preparative regimen, pre-hsct treatments administered, and disease status prior to the preparative regimen. Page 1 of 26 NOTE: Cytogenetic/Cytogenetics Cytogenetics is the study of chromosomes. Cytogenetic assessment involves testing blood or bone marrow for the presence of a known genetic abnormality that reflects the recipient s disease. Testing/reporting methods you may see include routine chromosome analysis (karyotyping), fluorescence in-situ hybridization (FISH), or microarray comparative genomic hybridization (acgh) testing. For more information about understanding cytogenetic testing and terminology, see Appendix R, Cytogenetic Abbreviations and Terminology. This form must be completed for all recipients whose primary disease is reported on Form 2000, question 9, as Acute Myelogenous Leukemia (AML), Acute Nonlymphocytic Leukemia (ANLL), other acute leukemia, or atypical CML (other leukemia). If the recipient had a Myelodysplastic/Myeloproliferative Syndrome (MDS/MPS) that transformed into AML prior to transplant, both Form 2010 (Acute Myelogenous Leukemia Pre-HSCT Data) and Form 2014 (Myelodysplasia/Myeloproliferative Disorders Pre-HSCT Data) must be completed. Key Fields Accuracy of the Key Fields is essential for ensuring that: Data are being reported for the correct recipient. Transplant centers have access to their data. Data are being shared with the correct donor center, cord blood bank, cooperative registry, or other agency. For instructions regarding the completion of the Key Fields, see Appendix K. Key fields include all fields listed in the box found in the upper right-hand corner of the first page of the paper form, or on the key page in the FormsNet TM application. Disease Assessment at Diagnosis Question 1: What was the date of diagnosis of Acute Myelogenous Leukemia? Report the date of the first pathological diagnosis (e.g., bone marrow biopsy) of AML. Enter the date the sample was collected for examination. If the diagnosis was determined at an outside center and no documentation of a pathological or laboratory assessment is available, the dictated date of diagnosis within a physician note may be reported. Do not report the date symptoms first appeared. Page 2 of 26 The date of diagnosis is important because the interval between diagnosis and HSCT is often a significant indicator for the recipient s prognosis post-hsct. If the exact pathological diagnosis date is not known, use the process described for reporting partial or unknown dates in General Instructions, Guidelines for Completing Forms. Question 2: Was this a secondary (therapy-linked) leukemia? (not MDS / MPS) Agents such as radiation or systemic therapy used to treat other diseases (e.g., Hodgkin lymphoma, non-hodgkin lymphoma, and breast cancer) can damage the marrow and lead to a secondary malignancy, such as AML. If the diagnosis of AML is therapy-related, check yes and continue with question 3. If the diagnosis of AML is not therapy-related, check no and continue with question 10. Do not answer this question yes if the recipient developed AML after an environmental exposure (e.g., exposure to benzene). Questions 3-4: What was the recipient s prior disease (malignant or nonmalignant): NOTE: Malignant vs. Non-malignant Malignant diseases involve cells dividing without control, which can spread to other parts of the body through blood and lymph systems. These diseases are usually characterized by unlimited, aggressive growth; invasion of surrounding tissues; and metastasis. Non-malignant diseases involve cell overgrowth, but lack the malignant properties of cancer. For example, a patient with rheumatoid arthritis is treated with methotrexate and then develops a secondary leukemia. Indicate the recipient s primary disease prior to the diagnosis of AML. If the recipient s prior disease is not listed, select other and specify the disease. Question 5: What was the date of diagnosis of prior disease? Report the date of the first pathological diagnosis (e.g., bone marrow or tissue biopsy) of the prior disease. Enter the date the sample was collected for examination. Do not report the date symptoms first appeared. This date must be prior to the AML diagnosis date entered in question 1. If the exact pathological diagnosis date is not known, use the process described for reporting partial or unknown dates in General Instructions, Guidelines for Completing Forms. Page 3 of 26 Questions 6-9: Specify treatment(s) for prior disease For each listed treatment, indicate yes, no, or unknown. If the treatment administered was Other treatment, specify the type of treatment given. Check all that apply; do not leave any responses blank. NOTE: In question 6, specify all chemotherapy drugs administered. In question 9, do not list individual chemotherapy drugs. Question 10: Did the recipient have a documented antecedent hematologic disorder (preleukemia or myelodysplastic syndrome)? AML often evolves from MDS or MPS. This transformation is typically distinguished by the percentage of blasts in the bone marrow. AML that transforms from MDS or MPS has a lower survival prognosis because of the association with unfavorable chromosomal abnormalities. AML can also evolve from Juvenile Myelomonocytic Leukemia (JMML). JMML is a rare form of chronic leukemia that affects young children, usually before the age of five. JMML results from DNA mutations in cells called monocytes. Normal monocytes attack invading microorganisms and assist lymphocytes in carrying out immune functions. Abnormal monocytes, or JMML cells, accumulate in the bone marrow and interfere with the production of normal white blood cells, red blood cells, and platelets. If there is documentation of an antecedent (prior) hematological disorder, check yes and continue with question 11. If MDS is suspected, but not confirmed by documented laboratory or pathologic findings, or if there is documentation of MDS concurrent with AML, check yes, MDS suspected and/or concurrent with AML diagnosis and continue with question 13. If the recipient does not have a documented antecedent hematologic disorder, check no and continue with question 13. Question 11: What was the date of diagnosis of antecedent hematologic disorder? Report the date of the first pathological diagnosis (e.g., bone marrow biopsy, laboratory results, or cytogenetic findings) of the antecedent hematologic disorder. Enter the date the sample was collected for examination. Do not report the date symptoms first appeared. If the source documentation does not include the exact pathological diagnosis date of the antecedent hematologic disorder, enter the AML diagnosis date in this field. Page 4 of 26 If the exact pathological diagnosis date is not known, use the process described for reporting partial or unknown dates in General Instructions, Guidelines for Completing Forms. Question 12: What was the classification of hematologic disorder at diagnosis? Indicate the classification of the hematologic disorder at diagnosis. Table 1. Myelodysplastic / Myeloproliferative Disorder Subtypes CLASSIFICATION DESCRIPTION Refractory Anemia (RA) Refractory Anemia with Ringed Sideroblasts (RARS) Refractory Anemia with Excess Blasts (RAEB-1) WHO classification terminology: Refractory Anemia with Excess Blasts- 1 (RAEB-1) Refractory Anemia with Excess Blasts in Transformation (RAEB-2) WHO classification terminology: Refractory Anemia with Excess Blasts- 2 (RAEB-2) Unilineage dysplasia in 10% of red blood cell precursors (i.e., erythroid dysplasia only) 5% blasts in the bone marrow 15% ringed sideroblasts Unilineage dysplasia in 10% of red blood cell precursors (i.e., erythroid dysplasia only) 5% blasts in the bone marrow 15% ringed sideroblasts in the bone marrow 5-9% blasts in the bone marrow and 5% blasts in the peripheral blood Or, if 5% blasts in bone marrow, then there must be 2-4% blasts in the peripheral blood No Auer rods Unilineage or multilineage dysplasia 10-19% blasts in the bone marrow And/or 5-19% blasts in the peripheral blood Auer rods may be seen Page 5 of 26 CLASSIFICATION Refractory Cytopenia with Multilineage Dysplasia (RCMD) Refractory Anemia with Ringed Sideroblasts with Dysplasia (RCMD- RS) DESCRIPTION 2 or more blood cytopenias Dysplasia in 10% in 2 or more myeloid lineages in bone marrow 5% blasts in the bone marrow No Auer rods 15% ringed sideroblasts Same criteria as RCMD except with 15% ringed sideroblasts WHO classification terminology: Refractory Anemia with Multilineage Dysplasia and Ringed Sideroblasts (RCMD-RS) 5q-Syndrome WHO classification terminology: Myelodysplastic Syndrome with Isolated Del(5q) Associated with macrocytic anemia often thrombocytosis, erythroblastopenia, megakaryocyte hyperplasia with nuclear hypolobation 5% blasts in bone marrow Deletion of part of the long arm (q arm) of chromosome 5 in bone marrow cells No Auer rods Page 6 of 26 CLASSIFICATION MDS Unclassifiable, Not Otherwise Specified (MDS-U) Chronic Myelomonocytic Leukemia (CMML) Examples: DESCRIPTION MDS that cannot be classified into defined category due to one or more atypical features 5% blasts in the bone marrow and Auer rods present Hypocellular MDS MDS with myelofibrosis Megakaryocyte dysplasia with fibrosis Refractory cytopenia with unilineage dysplasia (RCUD), or refractory cytopenia with multilineage dysplasia (RCMD) but with 1% blasts in blood MDS with unilineage dysplasia associated with pancytopenia Unequivocal dysplasia in 10% of cells in one or more myeloid cell lines accompanied by a chromosomal abnormality considered as presumptive evidence for a diagnosis of MDS (e.g., +8, -7, -5, etc.) 20% blasts (myeloblasts or monoblasts) in the peripheral blood or bone marrow 1 x10 9 /L monocytes in the peripheral blood No Philadelphia chromosome or BCR/ABL fusion gene present Dysplasia in one or more myeloid lineages. If dysplasia is absent or minimal, the diagnosis of CMML may still be made if other requirements are met. Page 7 of 26 CLASSIFICATION Chronic MPS Disorder, Not Otherwise Specified WHO classification terminology: Myeloproliferative neoplasm, unclassifiable (MPN, U) Chronic Neutrophilic Leukemia DESCRIPTION MPS that cannot be classified into defined category due to one or more atypical features Most cases of Myeloproliferative neoplasm, unclassifiable will fall into one of three groups: 1. Early stages of polycythemia vera (PV), primary myelofibrosis (PMF) or essential thrombocythemia (ET) in which the characteristics are not yet fully developed; 2. Advanced stage of MPN, in which pronounced myelofibrosis, osteosclerosis, or transformation to a more aggressive stage (i.e., increased blasts and/or dysplasia) obscures the underlying disorder; or, 3. Patients with convincing evidence of an MPN in whom a coexisting neoplastic or inflammatory disorder obscures some of the diagnostic clinical and/or morphological features. WBC 25x10 9 /L Persistent neutrophilia in peripheral blood (neutrophils + bands must be 80% of WBC) Myeloid hyperplasia in bone marrow (increased myeloid:erythroid ratio of 20:1 or greater) Hepatosplenomegaly Absence of the Philadelphia chromosome or BCR/ABL fusion gene Page 8 of 26 CLASSIFICATION DESCRIPTION Chronic Eosinophilic Leukemia Eosinophil count 1.5x10 9 /L 5-19% blasts in bone marrow No Philadelphia chromosome present Chronic Eosinophilic Leukemia and Hypereosinophilic Syndrome Polycythemia Vera (PCV) Hypereosinophilic Syndrome Elevated eosinophil count ( 1500 eosinophils/mm 3 ) persisting for at least six months without any known etiology Multiple organ damage (e.g., involvement of either the heart, nervous system, or bone marrow) No evidence of eosinophil clonality Overproduction of erythrocytes (red blood cells) by the bone marrow Diagnostic criteria: Increased hemoglobin ( 18.5 gm/dl in men & 16.5 gm/dl in women); presence of JAK2 mutation Bone marrow showing hypercellularity with trilineage growth (erythroid, granulocytic & megakaryocytic proliferation) Increased platelet or white blood count Low erythropoietin (EPO) level Page 9 of 26 CLASSIFICATION Chronic Idiopathic Myelofibrosis (with Extramedullary Hematopoiesis), Myelofibrosis with Myeloid Metaplasia, Acute Myelofibrosis or Myelosclerosis WHO classification terminology: Primary Myelofibrosis (PMF) Essential or Primary Thrombocythemia Juvenile Myelomonocytic Leukemia (JMML, JCML, JCMML) (no evidence of Ph 1 or BCR/ABL) DESCRIPTION Myeloproliferative syndrome of unknown etiology Myeloid proliferation Reactive fibrosis in the bone marrow Extramedullary hematopoiesis (formation and development of blood cells outside of the bone marrow) Presence of persistent (overproduction of platelets) thrombocytosis 450 x 10 9 /L without an alternative cause All of the following: No Philadelphia chromosome or BCR/ABL fusion gene Peripheral blood monocytosis 1 x 10 9 /L 20% blasts in the blood and bone marrow Two or more of the following: Hemoglobin F increased for age Immature granulocytes and nucleated red cells in the peripheral blood White blood cell count 1 x10 9 /L Clonal chromosomal abnormality GM-CSF hypersensitivity of myeloid progenitors in vitro Page 10 of 26 NOTE: If one of the first 15 options is selected for question 12, complete Form MDS in addition to Form AML. Form 2014 provides more detailed information on the preleukemic or myelodysplastic syndrome prior to the recipient developing AML. If the response to question 12 is juvenile myelomonocytic leukemia (JMML, JCML, JCMML) (no evidence of Ph 1 or BCR/ABL), complete Form JMML in addition to Form AML. Form 2015 provides more detailed information on the juvenile myelomonocytic leukemia prior to the recipient developing AML. Question 13: Did the recipient have a predisposing condition prior to the diagnosis of leukemia? A predisposing condition is a condition that contributes to the susceptibility of developing leukemia. Therefore, diagnosis of the condition increases the likelihood that the recipient will develop leukemia. If the recipient has a documented history of a predisposing condition, check yes and continue with question 14. If there is no history of a predisposing condition, check no and continue with question 16. NOTE: Aplastic Anemia and Fanconi Anemia If the response to question 14 is Aplastic Anemia, complete Form APL in addition to Form AML. This form provides detailed data specific to this disease. If the response to question 14 is Fanconi Anemia, complete Form 2029 FAN in addition to Form 2010 AML. This form provides detailed data specific to this disease. Questions 14-15: Specify condition Aplastic anemia is a condition in which the bone marrow does not produce enough new blood cells (red blood cells, white blood cells, or platelets). The cells produced are normal, but in insufficient amounts to maintain healthy numbers of blood cells. Bloom syndrome is a chromosomal disorder that is defined by many breaks and rearrangements in the chromosomes. It is characterized by growth deficiency and skin rash following exposure to the sun. Because of the greatly elevated rate of gene mutation, Bloom syndrome patients have a high risk of developing cancer. Page 11 of 26 Down syndrome is also a chromosomal disorder. It is characterized by an additional chromosome 21, also referred to as trisomy 21. Down syndrome patients exhibit a particular set of facial characteristics, growth deficiency, and cognitive impairment. Although Down syndrome patients have a reduced risk of developing many common malignancies, they have an increased risk of developing leukemia. Fanconi anemia is a rare genetic blood disorder that prevents the body from producing a sufficient number of new blood cells to function properly. Abnormal blood cells may also be produced. These patients are short in stature, exhibit skeletal anomalies, and have an increased risk of developing solid tumors and leukemias. Indicate the recipient s predisposing condition prior to the diagnosis of leukemia. If the condition was Other, specify the condition in question 15. Laboratory Studies at Diagnosis Report findings prior to any treatment of the primary disease for which the HSCT is being performed. Question 16: WBC Indicate whether the white blood count (WBC) is known or not known at the time of AML diagnosis. If known, report the laboratory value and unit of measure documented on the laboratory report. If not known, continue with question 17. Question 17: Blasts in blood Indicate whether the percentage of blasts in the peripheral blood is known or not known at the time of AML diagnosis. If known, report the percentage documented on the laboratory report. If not known, continue with question 18. NOTE: If a differential was performed and there were no blasts present in the peripheral blood, the laboratory report may not display a column for blasts. In this case, it can be assumed that no blasts were present and 0 can be entered on the form. The percentage of blasts in the peripheral blood may also be identified on a flow cytometry report. A flow cytometry report may be used as source documentation when reporting this data field. Page 12 of 26 Question 18: Blasts in bone marrow Indicate whether the percentage of blasts in the bone marrow is known or not known at the time of AML diagnosis. If known, report the percentage documented on the laboratory report at diagnosis. If not known, continue with question 19. NOTE: If the bone marrow pathology report states a range for blasts, enter the average of the range rounded to the nearest whole number (e.g., if 0-5%, enter 3%). If the report states 90% blasts, packed marrow, or sheets of blasts, enter 91% on the form. If the report states 5% blasts, enter 4% on the form. Question 19: Was extramedullary disease present at diagnosis? Extramedullary refers to disease found in organs or tissue outside the bone marrow or blood stream (e.g., central nervous system, testes, skin, soft tissue, etc.). Examples of extramedullary disease in AML patients include granulocytic sarcoma, subcutaneous nodules, leukemia cutis, and meningeal leukemia. If there is evidence of extramedullary disease at the time of diagnosis, indicate yes and continue with question 20. If there is no evidence of extramedullary disease at the time of diagnosis, indicate no and continue with question 25. Questions 20-24: Specify site(s) of disease Indicate the site of extramedullary disea
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