Leukemias and Myeloid Disoders 2

Question 1

Myeloproliferative diseases and syndromes

Answer 1

Clonal productive neoplastic proliferations with cell maturation

Marrow hyercellularity and splenomegaly

Elevated blood counts

JAK2 mutations often

Question 2

POlycythemia vera

Answer 2

Elevation in RBC mass increased platelet and granulocyte counts and basophilia

More males

Question 3

POlycythemia vera symptoms

Answer 3

Hypertension and poor platelet function
Enlargement of organs
Gradual transition to spent phase

Question 4

POlycythemia veria causes

Answer 4

JAK2 kinase
MUST have low EPO to distinguish from secondary polycythemia or relative polycythemia

Increased uric acid and histamine release

Question 5

Chronic ideopathic myelofibrosis

Answer 5

Marrow fibrosis related to PDGF and TGFbeta from abnormal megakaryocytes
Edxtramedulary heamtopoiesis with splenomegaly
Anemia with teardrops and leukoerythroblastic picture
JAK2 in 50-60%

Question 6

Essential thrombocytopneia

Answer 6

Sustained elevated platelet count
Thrombosis and hemorrhage probs

JAK2 50-60%

Question 7

Myelodysplastic syndromes

Answer 7

Usually refractory cytopenias which may precede AML
Changesi nbone marrow

Must have more than 20% blasts in bone marrow

Adults over 50

Pancytopenia

Rule out vitamin deficiency

Frequent cytogenic abnormalilty (5,7,8)

Question 8

Myelodysplastic/myeloproliferative dzs

Answer 8

Effective porliferation of one cell line with cytopenias of other

CMML - chronic myelomonocytic leukemia - overlap with monocytosis

Question 9

CLL/SLL age and what to look for in blood counts nad morphology

Answer 9

Over 50 - most common in adults in western hemisphere

B cell disorder with CD19, dim CD20, CD5, and CD23 co-expression and light chain restirction (all kappa or lmbda)

More than 000 peripheral lymphocytes, SLL if nodal presnetation and WBC not increased

Small mature lymphos with clumped chromatin

Question 10

Symptoms of CLL

Answer 10

LAD, hepatosplenomegaly 
IMmune dysfunction (positive coombs 15%, hypogammaglobulinemia 50%)

Question 11

Hairy cell leukemia

Answer 11

Chronic clonal mature B cell disorder
MUCH more in men 
Fried egg
Pancytopenia and monocytopenia 
Infections common
Splenomegaly

Question 12

Hairy cell leukemia immunophenotype

Answer 12

CD19, 20, right CD22, andf 11c, CD103, and CD25

Question 13

Hair cell prognosis

Answer 13

Drug therapy effective

2-CDA and pentostatin

Question 14

Neutropenia causes

Answer 14

Aplastic anemia
Bone marrow replacement
Drugs
Ineffective hematoposiesis (vit def)
Accerleared removal
Serious infections when count low

Question 15

Lymphopenia causes

Answer 15

Less common (HIV)

Question 16

Leukocytosis

Answer 16

Neutrophlia in infections, inflam, steroids
Eosino - allergies, parasites
Basophilia - renal failure, MPN
Monocytosis - TB, IBD, immune diorders

Question 17

Lymphocytosis with reactive lymphocytes think

Answer 17

virus

Question 18

Leukemoid reaction

Answer 18

High WBC count with immature granulocytes…differentiate from CML…no basophilia with leukemoid reactions

Question 19

Leukoerytrhoblastic rxn

Answer 19

Immature granulocytes and nucleated red cells

Question 20

Richter’s

Answer 20

Diffuse large B cell lymphoma (from CLL)

Question 21

Myelodysplastic syndromes can transform to

Answer 21

AML

Question 22

CLL cell markers and important

Answer 22

Clonally mature B cell (CD19,20) with CD5 and CD23

CD38 expression makes it worse

p53 deletion (17p) makes it worse as well