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MKSAP KEY POINTS > Heme/Onc KPs > Flashcards

Heme/Onc KPs Flashcards

1
Q

leads to disease control in 70% of adult patients with aplastic anemia?

A

cyclosporine and antithymocyte globulin

Immunosuppression

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2
Q

(9;22) gene translocation suggests? Treatment? Monitor this while on treatment?

A

CML;

TKR inhibitors (target BCR-ABL). Can prolong QT interval

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3
Q

Initial therapy for polycythemia vera?

When to add additonal therapy? (and what?)

A

ASA + phlebotomy

age>60 or high VTE risk - add hydroxyurea

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4
Q

General therapy for essential thrombocytopenia? Next med to add?

A

Observation

Platelet-lowering therapy (hydroxyura v anagrelide) if high risk

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5
Q

Treatment for primary myelofibrosis? unless?

A

Palliative (unless young - then HCT)

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6
Q

hypereosinophilic syndrome - treatment? If fails?

A

steroids; imatinib

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7
Q

defining clinical clue to promyelocytic leukemia? characterized by what mutation? Treatment?

A

DIC

t(15;17)

all-trans retinoic acid

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8
Q

Philadelphia chromosome–positive acute lymphoblastic leukemia can be treated with?

A

dasatinib (TKR)

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9
Q

most sensitive test for detecting monoclonal FLC gammopathies?

A

serum free LCs

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10
Q

Monoclonal gammopathy of undetermined significance is defined as?

If asymptomatic but otherwise does not meet this conditions?

A

M protein level less than 3 g/dL (or less than 500 mg/24 hr of urinary monoclonal free light chains), clonal plasma cells comprising less than 10% of the bone marrow cellularity, and the absence of plasma cell dyscrasia–related signs or symptoms

Smoldering multiple myeloma

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11
Q

Management of most patients with smoldering multiple myeloma?

A

CBC, Ca, Cr and M-protein assessment every 3-6 months

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12
Q

Best therapy for MM?

A

auto-SCT + melphalan after initial induction therapy

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13
Q 
Proteins in 
AL?
AA?
Hereditary?
Age-related?
Dialysis related?
A
  • Monoclonal free K/L LCs
  • transthyretin (TTR)
  • Serum amyloid A
  • TTR
  • B2 microglobulin
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14
Q

Assocaited for AL v AA amyloid?

A

AL - Plasma cell dyscrasias (MGUS, multiple myeloma), Waldenström

AA -Rheumatoid arthritis, inflammatory bowel disease, familial Mediterranean fever, chronic infection

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15
Q

Amyloid treated with SCT?

A

AL

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16
Q

Concern about Waldenstrom? Telltale symptoms?

A

hyperviscosity - headache, vision/hearing changes, dizziness, AMS

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17
Q

Dilated retinal veins, papilledema, and flame hemorrhages - think?

A

hyperviscosity syndromes (Waldenström or Type I cryoglobulinemia etc)

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18
Q

Normal trasferrin sat? Normal Ferritin?

A

<15; <12

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19
Q

Anemia in CKD - no diagnostic role for measuring?

A

erythropoietin

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20
Q

B12 v folate deficiency?

A

Methylmalonic acid and homocysteine are oth elevated

normal MMA, high HC

21
Q

Before starting folate treatment, need to rule out?

A

concomittant B12 deficiency (otherwise the increased hematopoisis can make patients have NS injury)

22
Q

chronic hemolytic anemia - need what supplementation?

A

folic acid

23
Q

bite cells and Heinz bodies?- suggest?

A

G6PD

24
Q

β-thalassemia v a-thalassemia - hemoglobin concentrations?

A

increased hemoglobin A2 levels and fetal hemoglobin levels vs normal

25
Q

disease-altering strategies in sickle cell disease?

A

HCT, transfusions, hydoxyurea

26
Q

mainstay treatments for warm autoimmune hemolytic anemia? Cold agglutinin disease?

A

Steroids

Coid avoidance (rituximab is fails)

27
Q

cause of cold agglutinin disease?

A

Pathogenic IgM molecules -> bind colder temperatures, typically in the fingers, toes, and nose

28
Q

ID causes of hemolytic anemia?

A

Malaria Plasmodium,
Babesia microti,
Clostridium perfringens, and
Bartonella bacilliformis

29
Q

Atypical hemolytic uremic syndrome? Effectively treated by?

A

congenital syndrome affecting complement activation (not preceeded by diarrheal illness)

eculizumab

30
Q

diagnostic test for ITP? Requires therapy when?

A

none; PLT<30 or bleeding

31
Q

heparin-induced thrombocytopenia - gold stardard for diagnosis?

A

serotonin release assay

32
Q

Diagnosis of von Willebrand disease?

A
  • von Willebrand factor antigen and activity <30%
  • low/normal factor VIII levels
  • Normal PT
  • High/normal PTT
33
Q

supranormal factor VIII levels seen in?

A

liver disease

34
Q

Never give alloimmunized patients?

A

erythrocytes containing antigens to which they are alloimmunized, even if repeat alloantibody screening is negative

35
Q

resh frozen plasma is ineffective for treating coagulopathies with an INR under?

A

1.85

36
Q

Inactivated 4-factor prothrombin complex concentrate contains? Give when?

A

II, VII, IX, X

Warfarin associated bleeding

37
Q

elayed hemolytic transfusion reactions occur? present with?

A

2-10 days after exposure

fever, jaundice, and worsening anemia

38
Q

Patients at high risk for anaphylactic transfusion reactions?

A

IgA deficiency

39
Q

transfusion-associated graft-verus-host disease - what is it? who is affected? Window of onset? Signs and symptoms? Prevention?

A

donor lymphocytes generate an immune response to recipient tissues

immunocompromised and unable to destroy the donor lymphocytes or in immunocompetent patients who are heterozygous for an HLA haplotype for which the donor is homozygous

up to 30 days after transfusion

Rash, jaundice, n/v/d, pancytopenia

Irradiation

40
Q

superficial venous thrombophlebitis treatment?

A

symptomatic therapy

41
Q

most common risk factor for Budd-Chiari syndrome?

A

myeloproliferative neoplasm (polycythemia vera, essential thrombocythemia, and primary myelofibrosis)

42
Q

diagnostic tests of choice for cerebral and sinus vein thrombosis?

Treatment?

A

CT venography and magnetic resonance venography

3 to 6 months if cerebral and sinus vein thrombosis was associated with a transient risk factor, 6 to 12 months if the event was unexplained and no higher risk thrombophilia has been detected, and long term if a higher risk thrombophilia is detected or the event is recurrent

43
Q

reverses anticoagulation with unfractionated heparin?

A

protamine

44
Q

Avoid LMWH if? Also avoid this anticoagulant?

A

CrCl<30; Fondaparinux

45
Q

patients taking warfarin with fluctuating INRs, daily supplementation this can stabilize the INR.

A

vitamin K

46
Q

When to evaluate gestational anemia?

A

Hgb<11 in first trimester

Hgb<10 in 2nd/3rd trimesters

47
Q

SIckle cell meds that must be discontinued in pregnancy?

A

Hydroxyurea, ACE inhibitors, and iron chelation therapy

48
Q

Goal PLT count in last month of pregnancy?

A

50 in case C-section is needed

49
Q

Highest risk of VTE during preggnancy is when?

A

6 weeks postpartum

MKSAP KEY POINTS (19 decks)

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