Leukemias and Myeloid Disoders 2 Flashcards

1
Q

Myeloproliferative diseases and syndromes

A

Clonal productive neoplastic proliferations with cell maturation

Marrow hyercellularity and splenomegaly

Elevated blood counts

JAK2 mutations often

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2
Q

POlycythemia vera

A

Elevation in RBC mass increased platelet and granulocyte counts and basophilia

More males

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3
Q

POlycythemia vera symptoms

A

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

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4
Q

POlycythemia veria causes

A

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

Increased uric acid and histamine release

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5
Q

Chronic ideopathic myelofibrosis

A

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

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6
Q

Essential thrombocytopneia

A

Sustained elevated platelet count
Thrombosis and hemorrhage probs

JAK2 50-60%

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7
Q

Myelodysplastic syndromes

A

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)

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8
Q

Myelodysplastic/myeloproliferative dzs

A

Effective porliferation of one cell line with cytopenias of other

CMML - chronic myelomonocytic leukemia - overlap with monocytosis

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9
Q

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

A

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

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10
Q

Symptoms of CLL

A
LAD, hepatosplenomegaly 
IMmune dysfunction (positive coombs 15%, hypogammaglobulinemia 50%)
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11
Q

Hairy cell leukemia

A
Chronic clonal mature B cell disorder
MUCH more in men 
Fried egg
Pancytopenia and monocytopenia 
Infections common
Splenomegaly
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12
Q

Hairy cell leukemia immunophenotype

A

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

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13
Q

Hair cell prognosis

A

Drug therapy effective

2-CDA and pentostatin

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14
Q

Neutropenia causes

A
Aplastic anemia
Bone marrow replacement
Drugs
Ineffective hematoposiesis (vit def)
Accerleared removal
Serious infections when count low
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15
Q

Lymphopenia causes

A

Less common (HIV)

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16
Q

Leukocytosis

A

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

17
Q

Lymphocytosis with reactive lymphocytes think

A

virus

18
Q

Leukemoid reaction

A

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

19
Q

Leukoerytrhoblastic rxn

A

Immature granulocytes and nucleated red cells

20
Q

Richter’s

A

Diffuse large B cell lymphoma (from CLL)

21
Q

Myelodysplastic syndromes can transform to

A

AML

22
Q

CLL cell markers and important

A

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

CD38 expression makes it worse

p53 deletion (17p) makes it worse as well