Module 7: Malignant Leukocyte Disorders Flashcards
Clinical findings in most leukocyte malignancies
Anemia (severe=myelophthisic)
Abnormal bleeding
Increased susceptibility to infection
Organ damage and failure
Four causes of malignancies
Chromosomal damage
Suppressed immune function
Genetic susceptibility
Trigger
Features of Chronic Myeloproliferative Disorders
Slow developing with repeated cellular reproduction
Cells are mostly mature
Leukoerythroblastic myelophthisic anemia
Extramedullary hematopoiesis
May evolve to different types or to acute
Common findings in Chronic Myelogenous Leukemia (CML)
Philadelphia chromosome WBC increased, usually >50 Left shift with whole granulocyte series Bone marrow markedly hypercellular (myeloid hyperplasia) Normocytic normochromic anemia LAP decreased
LAP stain increased or decreased in CML?
Decreased
Type of anemia common in CML
Normocytic normochromic
Polycythemia Rubra Vera (PRV) involves increased proliferation of:
Mainly erythroblasts, also granulocytes and megakaryocytes
Lab/clinical findings in PRV
RBCs very increased
PBS is leukoerythroblastic
BM hypercellular with large megakaryocytes
Extra medullary hematopoiesis and accompanying hepatosplenomegaly
Treated by venipuncture or myelosuppressive agents
Characteristics of Chronic Idiopathic Myelofibrosis (CIMF)
BM fibrosis Proliferation of fibroblasts which replace hematopoietic cells Leukoerythroblastic PBS Pancytopenia Dry taps
Lab findings to differentiate CML from CIMF
CIMF has increased LAP and no Philadelphia chromosome
Features of Essential Thrombocythemia (ET)
Rare primary megakaryocytic proliferation of unknown cause
PBS: thrombocytosis with megakaryocytes and pleomorphic platelets
BM: increased giant megakaryocytes
JAK2 gene mutation and associated MPDs
Val617Phe
PV (65-97%), ET (25-75%), CIMF (35-60%)
Features of Myelodysplastic Syndromes
Increased but ineffective and visibly abnormal hematopoiesis of erythrocytes, granulocytes, monocytes, and/or megakarocytes
Lab findings in Myelodysplastic Syndromes
Decreased RBC and reticulocyte counts
Macrocytosis
NRBCs
BM: erythroid hyperplasia with evidence of dyserythropoiesis
Features of Chronic Lymphocytic Leukemia (CLL)
Most common over age 50
Malignant proliferation of IgG secreting B cells
Results in incompetent mature lymphocytes
20% get AIHA
Lab findings in CLL
PBS: absolute lymphocytosis, smudge cells
BM: hypercellular with predominantly small lymphs
PAS positive in malignant lymphs but normal lymphs
Features of hairy cell leukemia
Variant of CLL
Splenomegaly, pancytopenia, hairy cells
Hairy cells are malignant mature B lymphs
TRAP positive
Plasma cell malignancies
Malignant proliferation of Ig producing cells, B lymphs, plasma cells
Increased concentrations of normal or abnormal antibodies in plasma
Multiple Myeloma/Plasma Cell Leukemia
Over production of single Ig class Excessive light chain production Hypergammaglobulinemia Abnormal M component on electrophoresis PBS: rouleaux, plasma cells
Heavy chain disease
Malignant cells secrete incomplete heavy chains (alpha, gamma, mu)
Alpha most common
Lymphomas
Malignant proliferation of lymphoid cells in lymphoid tissues
Become lymphocytic leukemias when large numbers of abnormal lymphs enter the blood
Non-Hodgkins lymphomas, how they are classified
Malignant proliferation of T or B cells
Cell size, morphology and type, proliferative pattern and intensity
Characteristics of Hodgkins lymphoma, requirement for diagnosis
Malignancy of mixed lineage
Reed-Sternberg cell
