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Internal Medicine > Heme > Flashcards

Heme Flashcards

1
Q

Ipilimumab/Yervoy

A

CTLA-4 immune checkpoint inhibitor

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2
Q

Nivolumab/Opdivo

A

PD-1 immune checkpoint inhibitor

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3
Q

Axicabtagene/Yescarta

A

anti-CD19 CAR-T (CD28 construct)

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4
Q

Brexucabtagene/Tecartus

A

anti-CD19 CAR-T (CD28 construct)

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5
Q

Tisagenlecleucel/Kymriah

A

anti-CD19 CAR-T (41BB construct)

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6
Q

Lisocabtagene/Breyanzi

A

anti-CD19 CAR-T (41BB construct)

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7
Q

Ciltacabtagene/Carvykti

A

anti-BCMA CAR-T (41BB construct)

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8
Q

Idecabtagene /Abecma

A

anti-BCMA CAR-T (41BB construct)

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9
Q

What does CTLA-4 bind to?

A

Dendritic cell ligand B7 (CD80), which would otherwise bind to CD28; this results in immune suppression

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10
Q

Which is more serious - PD-1 or CTLA-4 inhibition?

A

CTLA-4 has more severe side effects

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11
Q

Combined Research:
Cornell - Supplemental Application needed
UPenn - no supplemental needed
Mount Sinai - separate residency number
NYU - physician scientist track on website but link broken; clinical investigator track also exists
Yale - separate residency number
Beth Israel - direct ABIM
Mass Gen - Stanbury direct (fellowship not automatically coupled); will not automatically consider for categorical
Brigham - apply internally
Johns Hopkins - apply internally during intern year
Boston University - yes
Columbia - nothing
Montefiore/Einstein - not cancer specialty

A
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12
Q

Dabrafenib

A

BRAF inhibitor

Used in combination with trametinib, a MEK inhibitor, for melanoma and glioma

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13
Q

Cytarabine

A

Pyrimidine analog - competes with cytidine and incorporates into DNA, inhibiting DNA replication and inhibiting DNA polymerase

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14
Q

Nelarabine

A

Purine analog - metabolized into ara-GTP, which competes with guanosine

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15
Q

Anamnestic antibody response

A

Delayed hemolytic transfusion reaction to RBC antigen to which patient was previously sensitized (e.g. pregnancy)
Occurs days to weeks after transfusion

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16
Q

ABO incompatibility would cause what key features?

A
  1. Fever, flank pain, hemoglobinuria
  2. DIC
  3. Positive Coombs test
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17
Q

What does capillary refill assess?

A

Volume status/hypovolemia

Also look at dry mucous membranes, low skin turgor

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18
Q

What does pallor assess?

A

Anemia

Also look at mucous membranes, coarseness of hair, puffiness of face, nail defects

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19
Q

Potential exacerbations of sickle cell trait that can lead to intravascular hemolysis, tissue ischemia, and/or pain

A
  1. Flying at high altitude
  2. Dehydration due to alcohol
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20
Q

common cancers associated with malignant pericardial effusion

A

Breast
Lung
Lymphoma

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21
Q

Kerr sign

A

Referred pain from diaphragm and phrenic nerve to left shoulder - from splenic rupture

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22
Q

How is heparin-induced thrombocytopenia diagnosed?

A

Immunoassay (only if high titer)
Functional assay (serotonin release assay; gold standard)

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23
Q

Argatroban

A

Direct thrombin inhibitor

Used for HIT

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24
Q

Fondaparinux

A

Indirect inhibitor of Factor Xa (similar to LMWH), does not inhibit thrombin at all

Synthetic pentasaccharide sequence

Used for HIT

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25
Q

Chylothorax - signs and treatment

A

When lymphatic flow through thoracic duct is disrupted, leading to direct leakage of chyle into pleural cavity

Pleural fluid will contain T cells, Ig, and triglycerides (chylomicrons)

Treat with thoracentesis or chest tube, limiting dietary fat, and possible thoracic duct ligation

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26
Q

Sickle cell priapism treatment

A

Aspiration from corpora cavernosa, then intracavernous injection of phenylephrine

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27
Q

Who most often develops inhibitors of coagulation?

A
  1. Malignancy
  2. Rheumatic disease
  3. Postpartum period
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28
Q

Primary symptom of drug-induced thrombocytopenia

A

Oropharyngeal mucositis (sore throat, erythema, ulcers)

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29
Q

Smudge cells

A

Chronic lymphocytic leukemia

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30
Q

Waldenstrom macroglonulinemia - symptoms

A
  1. Hyperviscosity (diploma, hearing, headache)
  2. Neuropathy
  3. Hepatosplenomegaly, LAD

> 10% clonal B cells

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31
Q

Cyclophosphamide

A

Alkylating agent
Lupus with significant renal or CNS problems, vasculitis, cancer

Hemorrhagic cystitis, bladder cancer caused by acrolein metabolite - reduce risk with fluids, frequent voiding, mesna
Sterility, myelosuppression

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32
Q

What is a lung consequence of sickle cell disease?

A

Pulmonary hypertension due to vascular remodeling
Intravascular hemolysis releases heme, which consumes nitric oxide resulting in vasoconstriction; there is also inflammation and injury

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33
Q

Why is acute promyelocytic leukemia an emergency?

A

Tumor-induced consumptive coagulopathy - via activation of tissue factor (DIC) and increased generation of plasmin (fibrinolysis)

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34
Q

Tumor lysis syndrome - prophylaxis and treatment

A

Prophylaxis: IV fluids + xanthine oxidase inhibitor (allopurinol or febuxostat) or rasburicase
Treatment: IV fluids + rasburicase

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35
Q

Hydroxyurea main side effect

A

Myelosuppression

36
Q

How can CML be differentiated from leukemoid reaction?

A

CML has low leukocyte alk phos score since they are abnormal neutrophils

37
Q

CML typical mutation

A

BCR-ABL t(9;22)

38
Q

Paroxysmal nocturnal hemoglobinuria

A

Defect in anchor protein leading to complement-mediated hemolysis
Venous thrombosis, episodic intravascular hemolysis

39
Q

Eculizumab

A

Treatment for paroxysmal nocturnal hemoglobinuria

Anti-C5 antibody preventing complement activation

40
Q

Extravascular hemolysis causes

A

AIHA, intrinsic RBC enzyme defect (G6PD deficiency) or membrane defect (hereditary spherocytosis)

However, paroxysmal nocturnal hemoglobinuria, DIC, and cold agglutinin IgM are intravascular

41
Q

CVID comorbidities

A
  1. Autoimmune (RA, hemolytic anemia, pernicious anemia)
  2. IBD-like disease
  3. Enteropathy with makabsorption and weight loss
  4. Granulomas
  5. Increased risk of NHL
42
Q

What causes TRALI?

A

Donor anti-HLA or anti-granulocyte Ab cause granulocytes in lungs to agglutinate and degranulate

As opposed to febrile nonhemolytic, which is donor anti-HLA or cytokines

43
Q

What lab error can cause pseudothrombocytopenia?

A

Platelet clumping due to anti-EDTA antibodies

Drawing blood in heparin or citrate should resolve

44
Q

Sideroblastic anemia - effect on iron

A

Iron, ferritin, and transferrin saturation are all elevated, despite microcytic anemia

45
Q

Hodgkin treatment long-term adverse effects

A

Secondary malignancy
Cardiac disease
Radiation-induced hypothyroidism

46
Q

Folate is important for production of what nucleic acid?

A

Thymidine

47
Q

Azacytidine

A

Impairs DNA methylation

Used in MDS, AML

48
Q

Asplenic patients with pneumococcal infection are more likely to develop what heme manifestation?

A

Purpura fulminans:
DIC
Tissue thrombosis
Skin hemorrhage and necrosis

49
Q

How do neutrophils appear in B12 deficiency?

A

Hypersegmented

50
Q

What is the dysplastic appearance of neutrophils and erythrocytes in MDS?

A

WBC: Hyposegmented, hypogranular

RBC: Ovalomacrocytosis, normal or increased MCV

51
Q

Why does hereditary spherocytosis lead to gallstone risk?

A

Chronic hemolysis bilirubin leads to pigment gallstones (as well as jaundice and dark urine)

52
Q

Can splenectomy lead to platelet elevation in both short and long term?

A

Yes

53
Q

Ruxolitinib

A

Jak1/Jak2 inhibitor used in polycythemia vera, myelofibrosis

Watch out for platelet count

54
Q

Fludarabine

A

Purine analog

55
Q
A
56
Q

Heinz bodies, bite cells, blister cells

A

G6PD deficiency

Heinz bodies are denatured Hgb that may disappear later after episode of oxidative stress

57
Q

Anticoagulation in case of HIT

A

Argatroban

58
Q

Dabigatran reversal agent

A

Idarucizumab

59
Q

INR guidelines for warfarin

A

Bleeding: Discontine, give IV Vit K and PCC
INR >10 without bleeding: Discontinue, give oral Vit K
INR 4.5-10: Hold for few doses; can give low-dose Vit K
INR <4.5: Hold next dose, readjust maintenance

60
Q

Does cryo require ABO compatibility?

A

Yes

61
Q

Which vWD types are autosomal dominant?

A

Type 1 (mild-moderate quantitative deficiency)
Type 2 (qualitative problem)

Type 3 is complete absence of vWF

62
Q

What is more effective treatment for antiphospholipid antibody syndrome?

A

Warfarin

63
Q

TTP treatment

A

Plasma exchange

64
Q

When to treat ITP

A

Plt <30,000 or significant bleeding
Steroids and IVIG
2nd line: Splenectomy, rituximab, anti-D (Rh) Ig, TPO receptor agonist

65
Q

Basophilic stippling vs ringed sideroblasts

A

Basophilic stippling: Lead poisoning inhibits ribosomal RNA degradation

Ringed sideroblasts: Prussian blue-stained, iron-laden mitochondria at periphery

Lead poisoning can have both due to defective heme synthesis

66
Q

Sideroblastic anemia treatment

A

If acquired cause, give pyridoxine (cofactor of ALA synthase)

67
Q

Mentzer index

A

MCV/RBC: <13 is thalassemia, >13 is IDA

68
Q

Spherocytes can also be seen in…

A

AIHA

69
Q

PNH treatment

A

Prednisone
Eculizumab (complement C5 inhibitor - must give N. meningitidis vaccine)

BMT

70
Q

Warm vs cold causes of AIHA

A

Warm: lupus, CLL, lymphoma, various drugs
Cold: mycoplasma pneumoniae, EBV, Waldenstrom macroglobulinemia

71
Q

Warm AIHA treatment

A

Steroids, splenectomy if recurrent, or IVIG if severe and nonresponsive

72
Q

Blood smear - megaloblastic vs nonmegaloblastic difference

A

Nonmegaloblastic (alcohol, liver disease) doesn’t have neutrophil hypersegmentation

73
Q

Tea-colored urine

A

Porphyria cutanea tarda - caused by buildup of uroporphyrin

74
Q

Factors that exacerbate ALA synthase in acute intermittent porphyria

A

CYP450 inducers, alcohol, starvation

75
Q

Acute intermittent porphyria - signs and treatment

A

Abdominal pain, port-wine colored urine, polyneuropathy, psych

Treat with hemin and glucose

76
Q

Acute intermittent porphyria vs porphyria cutanea tarda - diagnosis

A

AIP: urine porphobilinogen
PCA: plasma or urine porphyrins

77
Q

What tumor lysis syndrome drug is contraindicated in G6PD deficiency?

A

Rasburicase

78
Q

Hairy cell leukemia initial treatment

A

Cladribine

79
Q

T15;17

A

APL

80
Q

T14;18

A

Follicular lymphoma - heavy chain Ig;BCL-2

81
Q

T8;14

A

Burkitt

82
Q

T11;14

A

Mantle cell

83
Q

Immunosuppressive/cytotoxic therapy fornhemophagocytix lymphohistiocytosis

A

Dexamethasone, etoposide

84
Q

Kit stain

A

Mast cells (mastocytosis); alsslo serum tryptase >20

85
Q

Langerhans cell histiocytosis - typical mutation

A

BRAF V600E

86
Q

Notable Langerhans cell histiocytosis symptoms

A

Diabetes insipidus and other hypothalamic-pituitary axis involvement, skin/oral brown purplish papules and/or erythematous rash, osteolytic bone lesions, and many others

Internal Medicine (23 decks)

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