Module 7: Malignant Leukocyte Disorders

Question 1

Clinical findings in most leukocyte malignancies

Answer 1

Anemia (severe=myelophthisic)
Abnormal bleeding
Increased susceptibility to infection
Organ damage and failure

Question 2

Four causes of malignancies

Answer 2

Chromosomal damage
Suppressed immune function
Genetic susceptibility
Trigger

Question 3

Features of Chronic Myeloproliferative Disorders

Answer 3

Slow developing with repeated cellular reproduction
Cells are mostly mature
Leukoerythroblastic myelophthisic anemia
Extramedullary hematopoiesis
May evolve to different types or to acute

Question 4

Common findings in Chronic Myelogenous Leukemia (CML)

Answer 4

Philadelphia chromosome
WBC increased, usually >50
Left shift with whole granulocyte series
Bone marrow markedly hypercellular (myeloid hyperplasia)
Normocytic normochromic anemia
LAP decreased

Question 5

LAP stain increased or decreased in CML?

Answer 5

Decreased

Question 6

Type of anemia common in CML

Answer 6

Normocytic normochromic

Question 7

Polycythemia Rubra Vera (PRV) involves increased proliferation of:

Answer 7

Mainly erythroblasts, also granulocytes and megakaryocytes

Question 8

Lab/clinical findings in PRV

Answer 8

RBCs very increased
PBS is leukoerythroblastic
BM hypercellular with large megakaryocytes
Extra medullary hematopoiesis and accompanying hepatosplenomegaly
Treated by venipuncture or myelosuppressive agents

Question 9

Characteristics of Chronic Idiopathic Myelofibrosis (CIMF)

Answer 9

BM fibrosis
Proliferation of fibroblasts which replace hematopoietic cells
Leukoerythroblastic PBS
Pancytopenia
Dry taps

Question 10

Lab findings to differentiate CML from CIMF

Answer 10

CIMF has increased LAP and no Philadelphia chromosome

Question 11

Features of Essential Thrombocythemia (ET)

Answer 11

Rare primary megakaryocytic proliferation of unknown cause
PBS: thrombocytosis with megakaryocytes and pleomorphic platelets
BM: increased giant megakaryocytes

Question 12

JAK2 gene mutation and associated MPDs

Answer 12

Val617Phe

PV (65-97%), ET (25-75%), CIMF (35-60%)

Question 13

Features of Myelodysplastic Syndromes

Answer 13

Increased but ineffective and visibly abnormal hematopoiesis of erythrocytes, granulocytes, monocytes, and/or megakarocytes

Question 14

Lab findings in Myelodysplastic Syndromes

Answer 14

Decreased RBC and reticulocyte counts
Macrocytosis
NRBCs
BM: erythroid hyperplasia with evidence of dyserythropoiesis

Question 15

Features of Chronic Lymphocytic Leukemia (CLL)

Answer 15

Most common over age 50
Malignant proliferation of IgG secreting B cells
Results in incompetent mature lymphocytes
20% get AIHA

Question 16

Lab findings in CLL

Answer 16

PBS: absolute lymphocytosis, smudge cells
BM: hypercellular with predominantly small lymphs
PAS positive in malignant lymphs but normal lymphs

Question 17

Features of hairy cell leukemia

Answer 17

Variant of CLL
Splenomegaly, pancytopenia, hairy cells
Hairy cells are malignant mature B lymphs
TRAP positive

Question 18

Plasma cell malignancies

Answer 18

Malignant proliferation of Ig producing cells, B lymphs, plasma cells
Increased concentrations of normal or abnormal antibodies in plasma

Question 19

Multiple Myeloma/Plasma Cell Leukemia

Answer 19

Over production of single Ig class
Excessive light chain production
Hypergammaglobulinemia
Abnormal M component on electrophoresis
PBS: rouleaux, plasma cells

Question 20

Heavy chain disease

Answer 20

Malignant cells secrete incomplete heavy chains (alpha, gamma, mu)
Alpha most common

Question 21

Lymphomas

Answer 21

Malignant proliferation of lymphoid cells in lymphoid tissues
Become lymphocytic leukemias when large numbers of abnormal lymphs enter the blood

Question 22

Non-Hodgkins lymphomas, how they are classified

Answer 22

Malignant proliferation of T or B cells

Cell size, morphology and type, proliferative pattern and intensity

Question 23

Characteristics of Hodgkins lymphoma, requirement for diagnosis

Answer 23

Malignancy of mixed lineage

Reed-Sternberg cell