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Heme Flashcards

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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
Chylothorax - signs and treatment
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
Sickle cell priapism treatment
Aspiration from corpora cavernosa, then intracavernous injection of phenylephrine
27
Who most often develops inhibitors of coagulation?
1. Malignancy 2. Rheumatic disease 3. Postpartum period
28
Primary symptom of drug-induced thrombocytopenia
Oropharyngeal mucositis (sore throat, erythema, ulcers)
29
Smudge cells
Chronic lymphocytic leukemia
30
Waldenstrom macroglonulinemia - symptoms
1. Hyperviscosity (diploma, hearing, headache) 2. Neuropathy 3. Hepatosplenomegaly, LAD >10% clonal B cells
31
Cyclophosphamide
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
What is a lung consequence of sickle cell disease?
Pulmonary hypertension due to vascular remodeling Intravascular hemolysis releases heme, which consumes nitric oxide resulting in vasoconstriction; there is also inflammation and injury
33
Why is acute promyelocytic leukemia an emergency?
Tumor-induced consumptive coagulopathy - via activation of tissue factor (DIC) and increased generation of plasmin (fibrinolysis)
34
Tumor lysis syndrome - prophylaxis and treatment
Prophylaxis: IV fluids + xanthine oxidase inhibitor (allopurinol or febuxostat) or rasburicase Treatment: IV fluids + rasburicase
35
Hydroxyurea main side effect
Myelosuppression
36
How can CML be differentiated from leukemoid reaction?
CML has low leukocyte alk phos score since they are abnormal neutrophils
37
CML typical mutation
BCR-ABL t(9;22)
38
Paroxysmal nocturnal hemoglobinuria
Defect in anchor protein leading to complement-mediated hemolysis Venous thrombosis, episodic intravascular hemolysis
39
Eculizumab
Treatment for paroxysmal nocturnal hemoglobinuria Anti-C5 antibody preventing complement activation
40
Extravascular hemolysis causes
AIHA, intrinsic RBC enzyme defect (G6PD deficiency) or membrane defect (hereditary spherocytosis) However, paroxysmal nocturnal hemoglobinuria, DIC, and cold agglutinin IgM are intravascular
41
CVID comorbidities
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
What causes TRALI?
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
What lab error can cause pseudothrombocytopenia?
Platelet clumping due to anti-EDTA antibodies Drawing blood in heparin or citrate should resolve
44
Sideroblastic anemia - effect on iron
Iron, ferritin, and transferrin saturation are all elevated, despite microcytic anemia
45
Hodgkin treatment long-term adverse effects
Secondary malignancy Cardiac disease Radiation-induced hypothyroidism
46
Folate is important for production of what nucleic acid?
Thymidine
47
Azacytidine
Impairs DNA methylation Used in MDS, AML
48
Asplenic patients with pneumococcal infection are more likely to develop what heme manifestation?
Purpura fulminans: DIC Tissue thrombosis Skin hemorrhage and necrosis
49
How do neutrophils appear in B12 deficiency?
Hypersegmented
50
What is the dysplastic appearance of neutrophils and erythrocytes in MDS?
WBC: Hyposegmented, hypogranular RBC: Ovalomacrocytosis, normal or increased MCV
51
Why does hereditary spherocytosis lead to gallstone risk?
Chronic hemolysis bilirubin leads to pigment gallstones (as well as jaundice and dark urine)
52
Can splenectomy lead to platelet elevation in both short and long term?
Yes
53
Ruxolitinib
Jak1/Jak2 inhibitor used in polycythemia vera, myelofibrosis Watch out for platelet count
54
Fludarabine
Purine analog
55
56
Heinz bodies, bite cells, blister cells
G6PD deficiency Heinz bodies are denatured Hgb that may disappear later after episode of oxidative stress
57
Anticoagulation in case of HIT
Argatroban
58
Dabigatran reversal agent
Idarucizumab
59
INR guidelines for warfarin
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
Does cryo require ABO compatibility?
Yes
61
Which vWD types are autosomal dominant?
Type 1 (mild-moderate quantitative deficiency) Type 2 (qualitative problem) Type 3 is complete absence of vWF
62
What is more effective treatment for antiphospholipid antibody syndrome?
Warfarin
63
TTP treatment
Plasma exchange
64
When to treat ITP
Plt <30,000 or significant bleeding Steroids and IVIG 2nd line: Splenectomy, rituximab, anti-D (Rh) Ig, TPO receptor agonist
65
Basophilic stippling vs ringed sideroblasts
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
Sideroblastic anemia treatment
If acquired cause, give pyridoxine (cofactor of ALA synthase)
67
Mentzer index
MCV/RBC: <13 is thalassemia, >13 is IDA
68
Spherocytes can also be seen in...
AIHA
69
PNH treatment
Prednisone Eculizumab (complement C5 inhibitor - must give N. meningitidis vaccine) BMT
70
Warm vs cold causes of AIHA
Warm: lupus, CLL, lymphoma, various drugs Cold: mycoplasma pneumoniae, EBV, Waldenstrom macroglobulinemia
71
Warm AIHA treatment
Steroids, splenectomy if recurrent, or IVIG if severe and nonresponsive
72
Blood smear - megaloblastic vs nonmegaloblastic difference
Nonmegaloblastic (alcohol, liver disease) doesn't have neutrophil hypersegmentation
73
Tea-colored urine
Porphyria cutanea tarda - caused by buildup of uroporphyrin
74
Factors that exacerbate ALA synthase in acute intermittent porphyria
CYP450 inducers, alcohol, starvation
75
Acute intermittent porphyria - signs and treatment
Abdominal pain, port-wine colored urine, polyneuropathy, psych Treat with hemin and glucose
76
Acute intermittent porphyria vs porphyria cutanea tarda - diagnosis
AIP: urine porphobilinogen PCA: plasma or urine porphyrins
77
What tumor lysis syndrome drug is contraindicated in G6PD deficiency?
Rasburicase
78
Hairy cell leukemia initial treatment
Cladribine
79
T15;17
APL
80
T14;18
Follicular lymphoma - heavy chain Ig;BCL-2
81
T8;14
Burkitt
82
T11;14
Mantle cell
83
Immunosuppressive/cytotoxic therapy fornhemophagocytix lymphohistiocytosis
Dexamethasone, etoposide
84
Kit stain
Mast cells (mastocytosis); alsslo serum tryptase >20
85
Langerhans cell histiocytosis - typical mutation
BRAF V600E
86
Notable Langerhans cell histiocytosis symptoms
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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