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Internal Medicine > Hematology > Flashcards

Hematology Flashcards

1
Q

Flow cytometry cell surface markers for PNH

A

CD55 & CD59 absent

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2
Q

PNH is associated with ____

A

thymoma

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3
Q

Things to send for pancytopenia

A
  1. bone marrow bx
  2. cytogenetic analysis (exclude MDS)
  3. flow cytometry
  4. vit b12, folate, hepatitis panel, HIV
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4
Q

Tx aplastic anemia

A
  1. stop causative agent if there is
  2. Immunosuppression (cyclosporine)
  3. Antithymocute globulin (if thymoma)
  4. Allogenic HSCT
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5
Q

PNH tx

A

eculizumab

Ppx anticoagulation
B12
folic acid
iron

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6
Q

Can you treat aplastic anemia with hematopoietic growth factors?

A

no

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7
Q

Diagnosis of pure red cell aplasia

A 
CT Chest (rule out thymoma) - usually T cell immune mediated
- Sometimes with Clonal CD57-positive (large granular lymphocytosis
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8
Q

Tx of pure red cell aplasia

A
  1. Transfusion, supportive, immunosuppressive agents (cyclosporin, prednisone, antithymocyte globulin.
  2. Thymectomy if thymoma
  3. IVIG for AIDS and Prior parvovirus19 infx
  4. Methotrexate or cyclosporine for large granular lymphocytosis
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9
Q

3 characteristics of Felty sd

A
  1. Neutropenia
  2. splenomegaly
  3. RA
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10
Q

MDS age prevalence and complication

A

> 60

Complications: acute leukemic syndromes or bone marrow failure

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11
Q

Dx of MDS

A
  1. Bone marrow: hypercellular marrow with dysplastic erythroid precursors
  2. Peripheral: cytopenia in at least 2 lines & morphologic abnormalitis of erythrocytes (MACROCYTOSIS, NUCLEATED ERYTHROCYTES, TEAR DROP);
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12
Q

Clonal abnormalities in MDS

A

Chromosome 3, 5, 7, 8, 17

-5q syndrome (has specific therapy)

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13
Q

Tx of MDS

A

Risk stratify with IPSS-R score

Low risk: no tx, infrequent transfusions
If transfusion: can give Erythropoieiss-stimulating agents (ESA)

High/very high risk: tx to prevent AML

  • Allogeneic HSCT 9younger pts)
  • Azacytidine and Decitabine

for -5q syndrome: LENALIDOMIDE

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14
Q

What do you use Azacytidine and Decitabine for

A

MDS with -5q syndrome

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15
Q

CML Dx

A

t(9;22) - Philadelphia Chromosome

BCR-ABL gene

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16
Q

Clinical features of CML

A

splenomegaly, elevated WBC, increased granulocytes in all phases of maturation peripherally

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17
Q

Criteria for blast or accelerated phase of CML

A

> 10%

Accelerated = >20 % of blasts

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18
Q

Tx for CML

A
  1. Hydroxyurea (palliative)
  2. Tyrosine kinase inhibitors (Imitinib, dasatinib, nilotinib) - long term dz control
  3. Allogenic HSCT - potential cure
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19
Q

Side effect of all tyrosine kinase inhibitors

A

prolonged QT-interval

20
Q

Definition of essential thrombocythemia and common mutation

A

> 450,000 in the absence of secondary causes of reactive thrombocytosis (IDA, bleeding, cancer, infection, chronic inflammatory disease)

JAK2

Can have vasomotor sx like pain

21
Q

Tx of essential thrombocythemia for

  1. low-risk patients (<60 years, no previous thrombosis, leukocyte count <11,000)
  2. high risk non-pregnant patients
  3. Lifethreatening (TIA, stroke, MI, GI bleed)
A
  1. low-dose ASA
  2. HR: + hydroxyurea
  3. Plateletpharesis
22
Q

Other causes of high Hb other than polycythemia vera

A

Ectopic EPO (renal cell carcinoma or other kidney disease)
hypoxemia
exogenous androgen

23
Q

Dx PCV

A
  1. EPO (should be low in setting of erythrocytosis)
  2. JAK2 mutation testing
  3. UA (microscopic hematuria from EPO-producing hypernephroma)
24
Q

Tx PCV

A

phlebotomy to goal Hct <45%
+ hydroxyurea
Low dose ASA

25
Q

When should you absolutely consider PCV

A

Budd-chiari syndrome (hepatic vein thrombosis)

Portal vein thrombosis

26
Q

Characteristics of primary myelofibrosis (4)

A
  1. Massive Splenomegaly and hepatomegaly (extramedullary hematopoiesis)
  2. Normocytic Anemia
  3. Circulating erythroblast & myeloid precursors, giant platelets, teardrop erythrocytes
  4. Bone marrow fibrosis
27
Q

Causes of death in patients with primary myelofibrosis (3)

A

Bone marrow failure,
transformation to acute leukemia,
portal HTN

28
Q

Tx of primary myelofibrosis

A
  1. Supportive
  2. Hydroxyurea & RUxolitinib (JAK2 inhibitor) - decreases splenomegaly
  3. Allogenic HSCT (pts <60 yo)
29
Q

Hypereosinophilic syndrome definition

A

> 1500

30
Q

Tx of HES with mutation of PDGFR

A

imitinib

31
Q

Up to ___% of patients with ALL have CNS involvement. Why is this important.

A

30!

Need intrathecal chemo with induction therapy

32
Q

AML vs. ALL

A

Significant issues with thrombocytopenia, bleeding, bruising, petechiae, infection

NO lymphadenopathy or hepatosplenomegaly

33
Q

Findings that are pathognomonic to AML

A

Auer rods on peripheral smear

+/- gingival hypertrophy and leukemia cutis (violaceous, nontender, cutaneous plaques)

34
Q

Dx of AML

A

Bone Marrow Bx >20% myeloblasts

35
Q

Risk stratification AML cytogenetic

A

Favorable: t (8;21), inv (16), t(15;17)

High Risk: complex genetic abnormalities (>5 abnl -5, -7, -5q, 3q)

36
Q

APML cytogenetic and tx

A

t(15;17)

All-trans retinoic acid (ATRA)

37
Q

Risk of ATRA and treatment

A

differentiation syndrome (fever, pulmonary infiltrates, hypoxemia, hyperleukocytosis)

Tx: dexamethasone

38
Q

WBC cutoff for leukapharesis

A

> 50k

39
Q

AML tx if HSCT not available (older pt)

A

Anthracyclines
Cytarabine (Ara-C)
Azacitidine
Decitabine

40
Q

IgM gammopathy is associated with ___

A

B- cell lymphoma

41
Q

What other studies should you also get with IgM gammopathy

A

CT Chest, abd, pelvis

42
Q

MGUS findings (3)

A

serum monoclonal protein <3 g/dL
Bone marrow clonal plasma cell <10%
No end-organ damage

43
Q

Smoldering MM

A

serum monoclonal protein >3
Bone marrow clonal plasma cells >10%
No end-organ damage

44
Q

MM requiring therapy

A

Serum monoclonal protein present
Bone marrow clonal plasma cells >10%
End-organ damage present (CRAB mnemonic)

45
Q

Treatment of MM

A 
Proteosome inhibitor (bortezomib)
Immunomodulatory agent (thalidomide or lenalidomide)
Glucocorticoids
Ankylating agent (mephalan or cyclophosphamide) for non-transplant candidates
46
Q

Risk of bortezomib and thalidomide

A

peripheral neuropathy

Internal Medicine (6 decks)

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