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Esther's Step 1 Topics > Heme Onc > Flashcards

Heme Onc Flashcards

1
Q

Lymphoid stem cells give rise to

A

B lymphocytes, T lymphocytes, lymphocytic dendritic cells, NK cells

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2
Q

Myeloid stem cells give rise to

A

Erythrocytes, megakaryocytes, granulocytes (neutrophils, basophils, eosinophils), monocytes (macrophages), mast cells, myeloid dendritic cells

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3
Q

RBCs have what kind of antiporter

A

Bicarb/Cl-. Allows RBCs to carry CO2

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4
Q

HBA2

A

Alpha2delta2

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5
Q

HbF

A

Alpha2gamma2

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6
Q

Hb Barts

A

Gamma4

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7
Q

HbH

A

Beta4

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8
Q

Lead poisoning? Sx and treatment

A

L - Lead lines on gingivae and metaphyses on bone xray, E - encephalopathy, erythrocyte basophilic stippling, A-abdominal colic and sideroblastic anemia D - wrst/food drop DEMERCAPROL and EDTA 1st line treatment. Succimer used for kids

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9
Q

ALA synthase defect what kind of inheritance

A

X linked

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10
Q

What is aplastic anemia?

A

Pancytopenia - thrombocytopenia, leukopenia, severe anemia. Normal cell morphology but hypocellular bone marrow with fatty infiltration. Caused by failure of destruction of myeloid cells due to 1) drugs (chloramphenicol, alkylating agents, benzene, antimetabolits) 2) viral agents (EBV, Parvovirus B19, HIV, HCV), Fanconi anemia (DNA repair defect). NO SPENOMEGALY

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11
Q

Hemoglobinuria (dark urine) and back pain after oxidant stress suggestive of? Examples of stressors

A

G6PD deficiency x linked recessive. Oxidant stressors:sulfa drug (dapsone), fava beans, anti-malarials, infections

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12
Q

Paroxysmal nocturnal hemoglobinuria is what? Triad? Labs? Tx

A

Increased complement mediated RBC lysis due to defect in GPI anchor for decay acclerating factor that normally protects RBCs from complement attack. Triad: Coombs negative hemolytic anemia, thrombosis, PANCYTOPENIA. Labs: CD55/59 NEGATIVE RBCs on flow cytometry. Tx: Eculizumab

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13
Q

Sickle cell anemia defect? HBC defect

A

A/R. Single amino acid replacement. Beta globin gene. 6 position 1) gluatmic acid to valine 2) glutamic acid to lysine

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14
Q

Hemolytic Uremic Syndrome? Caused by? Triad?

A

Triad: Acute renal failure, thrombocytopenia microangiopathic hemolytic anemia. Caused by drugs or infection. O157:H7 dysentery. andE coli verotoxin

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15
Q

Thrombotic Thrombocytopenic purpura? Caused by? Sx? Tx?

A

Defiency or inhibition of ADAMTS 13 which is vWF metalloprotease => decreased degradation of vWF multimers => more platelet adhesion=> platelet aggrgstion and thombosis. Leads to DECREASED PLATELET COUNT AND increased bleeding time. Labs: Schistocytes and increased LDH. Symptoms: Neuro and Renal dysfunction, fever, thrombocytopenia, microangiopathic hemolytic anemia

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16
Q

What test is used to diagnose Von willenbrand disease?

A

Ristocetin cofactor Assay. Normally, ristocetin induces platelet aggregation but lack of vWF will not cause this.

17
Q

Ppl with protein C and S deficiency are at greater risk for?

A

Thrombotic skin necrosis with hemorrhage following administration of warfarin

18
Q

Reed Sternberg cells are characteristic for? What do they look like? Cell markers?

A

Hodgkins Lymphoma. Looks like two owl eyes. CD15+ CD30+ B cell origin

19
Q

Hodgkins Lymhoma - what is most common? What form has stronger prognosis? 50% a/w

A

Nodular sclerosing (affects both men and women equally, all other forms affects men more). Rich lymphocytic reaction => better prognosis. EBV

20
Q

G6PD deficiency inheritence

A

X linked recessive

21
Q

What are the neoplasms of mature B cells (Non-Hodgkin)? Which is most common?

A

1) Diffuse large cell B lymphoma (most common) 2) Burkitt lymphoma 3) Follicular lymphoma 4) Mantel cell lymphoma

22
Q

Translocation for Burkitt lymphoma

A

T(8;14) - translocation of c myc (8) and heavy chain (14). Overexpression of cmyc=> cell growth. A/W EBV. Starry sky appearance of sheets of lymphocytes with scattered macrophages

23
Q

Translocation for diffuse large cell

A

t (14;18)

24
Q

Translocation for mantle cell

A

Older males t (11;14) translocation of cyclin D1 (11) and heavy chain (14). CD5+

25
Q

Translocation for follicular lymphoma

A

Adults. T(14;18) - translocation of heavy chain 14 (14) to bcl-2. “waxing nad waning”

26
Q

HLTV-1 causes?

A

Adult T cell l ymphoma - adults presen tiwth cutaneous lesions - japan, west africa, carribbean

27
Q

Mycosis fungoides/Sezary syndrome

A

Adults present with cutaneous patches/plaques/tumors with potential to spread to lymph nodes and viscera

28
Q

Multiple myeloma produces large amounts of what Igs? Histo smear?

A

IgG (55%) and IgA (25%). Smear: fried egg appearace. Plasma cells with large eccentric nuclei

29
Q

With MM think of what clues?

A

M protein spike, hypercalcemia, renal insufficiency, anemia, bone pain/bone lytic lesions, rouleax formation of RBCs, Ig light chains in urine (bence join proteins).

30
Q

What are the lymphoid leukemias

A

1) Acute lymphoblastic leukemia 2) small lymphocytic leukemiac/chronic lymphocytic leukemia 3) hairy cell leukemia

31
Q

What are the myeloid leukemias

A

1) AML (important M3 promyelotic leukemia) 2) CML

32
Q

ALL? Age? Markers? May spread where? Which translocation type has better prognosis? Smear?

A

lymphoid leukemia. Age:<15 years old. T-ALL can present as mediastinal mass with dysphagia and superior vena cava syndrome. A/w down syndrome. TdT+(marker of pre B and pre T cells), CD10+ in pre-B Cells only. May spread to CNS and tests. T(12;21) was better prognosis. Smear = increased lymphoblasts in periphery and bone marrow

33
Q

Small lymphocytic lymphoma/chronic lymphoctic leukemia Age? Smear?

A

> 60 years old. Cd20+, CD5+ b celll neoplasm. Often asymptomatic. Autoimmune hemolytic anemia can occur. Smear shows “smudge cells”. Involves lymph nodes.

34
Q

Hairy cell leukemia. Age. Stains?

A

Rare lymhpid neoplasm. Adults. Mature B cell tumor in elderly. Filamentous projections.Stains TRAP + although large replaced with flow cytometry. Tx: cladibrine which is an adenosine analog that inhibits adenosine deaminase.

35
Q

Translocation for M3 type of AML

A

t(15;17) response to all-trans retinoic acid

36
Q

AML - age? Stains? RF? Common presentation? Tx?

A

65 years, stains peroxidase +, see Auer rods, increased circulating myeloblasts on peripheral smear. RF: prior exposure to alkylating chemotherapy, down syndrome. Common ppt of DIC and can be induced by chemotherpay due to release of Auer rods. M3 subtype responds to trans retinoic acid which promotes differentiation of the blasts

37
Q

translocation for CML? Smear?

A

t(9;22) fused bcrl-able. Myeloid stem cell proliferation. Increased neutrophils, metamyelocytes and basophils. Very low leukocyte alk phos. Responds to imatinib

38
Q

Birbeck granules S-100a

A

Langerhans histiocytosis. Recurrent otitits media, skin rash - child has lytic bone lesion.

Esther's Step 1 Topics (13 decks)

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