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

24
Q

Fondaparinux

A

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

Synthetic pentasaccharide sequence

Used for HIT

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

26
Q

Sickle cell priapism treatment

A

Aspiration from corpora cavernosa, then intracavernous injection of phenylephrine

27
Q

Who most often develops inhibitors of coagulation?

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

Primary symptom of drug-induced thrombocytopenia

A

Oropharyngeal mucositis (sore throat, erythema, ulcers)

29
Q

Smudge cells

A

Chronic lymphocytic leukemia

30
Q

Waldenstrom macroglonulinemia - symptoms

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

> 10% clonal B cells

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

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

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)

34
Q

Tumor lysis syndrome - prophylaxis and treatment

A

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

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