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Step 1 Flash Cards > Heme Onc > Flashcards

Heme Onc Flashcards

1
Q

platelet granules

A
  • dense granules contain ADP and Ca

- alpha granules contain vWF, fibrinogen

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2
Q

monocytes

A
  • differentiates into macrophages in the tissues
  • large, kidney shaped nucleus
  • extensive “frosted glass” cytplasm
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3
Q

macrophage

A
  • differentiate from circulating blood monocytes
  • activated by gamma-interferon
  • CD 14 cell surface marker
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4
Q

eosinophils

A
  • defends against helminths

- produces histaminidase and arylsulfatase (limits reaction following mast cell degranulation)

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5
Q

basophil

A
  • mediates allergic reaction

- granules contain heparin, histamine and leukotrienes

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6
Q

mast cell

A
  • IgE cross-links upon antigen binding, causing degranulation which releases histamine, heparin and eosinophil chemotactic factor
  • involved in Type I HS reactions
  • systemic mastocytosis: mast cell proliferation in the BM and other organs (increased gastric acid 2/2 histamine)
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7
Q

dendritic/Langerhans cells

A
  • MHC Class II and Fc receptor on surface
  • APCs
  • Langerhans cells in the skin have “racket shaped” intracytoplasmic granules- Birbeck granules
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8
Q

platelet plug formation cascade

A
  • injury causes subendothelial collagen to expose vWF
  • adhesion of platelets to vWF via GpIb receptor
  • platelet degranulation releases ADP and Ca, which causes platelets to expose GPIIb/IIIa receptors
  • fibrinogen binds GpIIb/IIIa receptors –> platelet aggregation
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9
Q

increased ESR seen in

A
  • infections, AI disease, malignant neoplasms, GI disease like UC, pregnancy
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10
Q

decreased ESR seen in

A
  • polycythemia, sickle cell, CHF, microcytosis, hypofibrinogenemia
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11
Q

acanthocytes indicative of

A
  • liver disease, abetalipoproteinemia
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12
Q

basophilic stippling indicative of

A
  • anemia of chronic disease, Etoh abuse, Lead poisoning, Thalassemias
  • Basically, ACiD alcohol is LeThal
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13
Q

schistocyte/helmet cells indicative of

A
  • DIC, TTP/HUS, traumatic hemolysis (mechanical heart valve)
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14
Q

tear drop cell indicative of

A
  • bone marrow infiltration (myelofibrosis)
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15
Q

target cell indicative of

A
  • HbC disease, Asplenia, Liver disease, Thalassemia

- “HALT” said the hunter to his target

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16
Q

iron deficiency anemia labs

A

decrease iron, inc TIBC, low ferritin, inc RDW

  • can have pitted nails
  • increased free protoporphyrin because heme = iron + protoporphyrin and you are not making enough iron
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17
Q

Plummer-Vinson syndrome

A
  • a manifestation of iron deficiency anemia

- triad: Fe def anemia, esophageal webs (dysphagia) and atrophic glossitis (beefy red tongue)

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18
Q

alpha thalassemia

A
  • cis deletion in Asians, trans deletion in Africans
  • 4 allele deletion - hydrops fetalis
  • 3 allele deletion - HbH disease
  • 2 allele deletion - no clinically significant anemia
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19
Q

beta thalassemia minor

A
  • point mutations at splice sites and promoter sequences lead to decreasedB globin synthesis
  • prevalent in mediterranean populations
  • B chain is underproduced
  • usually asymptomatic
  • diagnose with increased HbA2 (< 3.5%) on electrophoresis
  • carrier status carries protection from plasmodium falciparum malaria
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20
Q

beta thalassemia major

A
  • homozygote mutation, B chain is absent –> severe anemia requiring blood transfusion
  • marrow expansion leads to skeletal deformities (“chipmunk” facies)
  • increased risk of parvo B19 aplastic anemia
  • HbF is protective, so symptoms show up at 6 months
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21
Q

HbS/B thalassemia heterozygote

A
  • mild to moderate sickle cell disease depending on the amount of B globin production
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22
Q

lead poisoning

A
  • inhibits ferrochelatase and ALA dehydratase –> decreased heme synthesis and inc RBC protoporphyrin
  • LLEEAADD: Lead Lines, Encephalopathy and Erythrocyte basophilic stippling, Abdominal colic and sideroblastic Anemia, Drops (foot and wrist drop) and Dimercaprol/EDTA for treatment
  • give succimer to kids cause it “sucks” to be a kid with lead poisoning
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23
Q

sideroblastic anemia

A
  • defect in protoporphyrin synthesis (heme = Fe + protoporphyrin, so defect in heme synthesis)
  • can be hereditary defect in ALA synthase or aquired myelodysplastic syndromes or reversible (alcohol, lead, B6 def, copper def, isoniazid)
  • Fe enters the mitochondria to form heme, but there is no protoporphyrin to combine with –> Fe laden macrophages around the nucleus
  • increased iron, normal TIBC, inc ferritin
  • treat with pyroxidine
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24
Q

Schilling Test

A
  • enteric B12 + IM B12 - if increased in urine, diagnoses deficiency
  • enteric B12 + IF - if increased in urine now, then its a IF deficiency
  • if it never increases in the urine, its malabsorption (pancreatic insufficiency, ileal disease, or SIBO)
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25
Q

orotic aciduria

A
  • cannot convert orotic acid to UMP (defect in UMP synthase)
  • AR
  • megaloblastic anemia non-responsive to folate or B12
  • no hyperammonemia
  • treat with uridne monophosphate to bypass mutated gene
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26
Q

intravascular hemolysis

A
  • decreased haptoglobin, increased LDH, schistocytes and reticulocytes, urobilinogen in urine
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27
Q

extravascular hemolysis

A
  • spherocytes, increased LDH + increased unconjugated bili
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28
Q

anemia of chronic disease

A
  • inflammation causes the liver to release hepcidin, which binds ferritin and suppresses EPO
  • low iron, low TIBC and high ferritin, increased free erythrocyte protoporphyrin
  • treat the primary disease and some people get EPO (esp cancer pts)
29
Q

G6PD

A
  • most common enzymatic disorder of RBCs
  • X linked recessive
  • defect in G6PD leads to decreased glutathione, increased RBC susceptibility to oxidative stress and hemolysis
  • back pain (Hgb is nephrotoxic), hemoglobinuria
  • Heinz bodies and bite cells
30
Q

pyruvate kinase deficiency

A
  • AR defect in pyruvate kinase
  • decreased ATP and rigid RBCs
  • hemolytic anemia of the newborn
31
Q

Hgb C defect

A
  • glutamic acid to lysine mutation at residue 6 in B globin
  • Hgb C crystals seen in smear
  • will no travel as far on electrophoresis because there is decreased total negative charge
  • Pts with HbCS have milder disease than Hb SS
32
Q

PNH

A
  • inreased complement mediated lysis of RBCs due to lack of GPI anchor for factors that prevent from complement destruction
  • resp acidosis at night causes complement mediated RBC lysis –>coombs negative hemolytic anemia, pancytopenia, and venous thrombosis
  • CD55/59 negative cells - acquired mutation in the precursor cell
  • treat with eculizumab
  • increased incidence of AML
33
Q

Warm agglutinin hemolytic anemia

A
  • IgG mediated, can be seen with SLE, CLL and certain drugs (penicillin, methyldopa)
  • coombs +ve
34
Q

cold agglutinin hemolytic anemia

A
  • IgM mediated, acute anemia triggered by cold, seen in CLL, mycoplasma pneumonia, and infectious mono
  • coombs +ve
35
Q

lead poisoning

- enzymes affected, accumulated substrate and presenting symptoms

A
  • ALA synthase and ferrochelatase
  • protoporphyrin, d-ALA
  • microcytic anemia, GI and kidney disease
36
Q

acute intermittent porphyria

- enzyme affected, accumulated substrate and presenting symptoms

A
  • porphobilinogen deaminase
  • porphobilinogen, d-ALA, coporphobilinogen (urine)
  • symptoms (5 P’s): painful abdomen, port-wind colored urine (gets darker when exposed to air), polyneuropathy, psychological disturbances, precipitated by drugs, alcohol and starvation
  • treat with glucose and heme, which inihibit ALAS
37
Q

porphyria cutanea tarda

- enzyme affected, accumulated substrate and presenting symptoms

A
  • uroporphyrinogen decarboxylase
  • uroporphyrin (tea colored urine)
  • blistering cutaneous photosensitivity
  • most common porphyria
38
Q

Bernard-Soulier syndrome

A
  • dec platelet count, increased bleeding time

- defect in platelet plug formation due to decreased Gp1b –> defect in platelet-vWF adhesion

39
Q

Glanzmann thrombasthenia

A
  • increased bleeding time
  • defect in platelet plug formaiton due to decreased GpIIb/IIIa –> defective platelet aggregation
  • blood smear shows no platelet clumping
40
Q

immune thrombocytopenia

A
  • dec platelet count, increased bleeding time
  • anti-GpIIb/IIIa Abs –> splenic macrophage consumption of platelet/Ab complex
  • may be triggered by viral illness, decreased platelet survival
  • increased megakaryocytes on BMB
41
Q

TTP

A
  • inhibition or deficiency of ADAMTS 13 (vWF metalloproteinase) –> decreased degradation of vWF multimers
  • large multimers increase platelet adhesion and increase platelet aggregation and thrombosis
  • decreased platelet survival
  • labs show schistocytes and increased LDH
  • sympt (pentad): neurologic and renal symptoms, fever, thrombocytopenia, and microangiopathic hemolytic anemia
  • treat with exchange transfusion and streroids
42
Q

vWF disease

A
  • increased bleeding time with normal or increased PTT
  • vWF protects factor 8
  • mild inherited bleeding disorder , AD
  • diagnosed with ristocetin cofactor assay (decreased agglutination is diagnostic)
  • treat with DDAVP
43
Q

Factor V Leiden

A
  • factor V resistant to inhibition by activated protein C, so adding protein C will not change the normal PTT
  • most common cause of hypercoaguability in whites
44
Q

prothrombin gene mutation

A
  • mutation in the 3’ untranslated region –> increased production of prothrombin –> increased plasma levels and venous clots
45
Q

antithrombin deficiency

A
  • antithrombin deficiency - doesnt have any direct effect on PT/PTT, but PTT will not increase as much with heparin administration
46
Q

protein C or S deficiency

A
  • decreased ability to inactivate factors V and VIII

- increased risk of thrombotic skin necrosis with hemorrhage following coumadin

47
Q

leukemoid reaction vs. CLL

A
  • leukemoid reaction will have increased ALP

- CLL will have decreased ALP

48
Q

Reed-Sternberg cells

A
  • seen in Hodgkin lymphoma
  • “owl eye” nuclei, CD15 and CD30+ (2 owl eyes x 15 = 30)
  • nodular sclerosing type most common
  • lymphocyte-rich forms have better prognosis
49
Q

Burkitt Lymphoma

A
  • occurs in adolescents and young adults
  • t(8;14) - translocation of c-myc and heavy chain IgG
  • starry sky appearance, sheets of lymphocytes with interspersed macrophages
  • med sized lymphocytes with high proliferation ( high KI-67 fraction)
  • associated with EBV
  • jaw lesion in endemic form in Africa, pelvis and abdomen in sporadic form
50
Q

diffuse large B cell lymphoma

A
  • usually older adults, 20% kids
  • t (14;18)
  • most common type of non-Hodgkin lymphoma in adults
51
Q

mantle cell lymphoma

A
  • older males
  • t (11;14) translocation of cyclin D1 and heavy chain IgG
  • CD5+
52
Q

follicular cell lymphoma

A
  • adults
  • t(14;18) traslocation of heavy-chain Ig and Bcl-2
  • indolent course
  • bcl2 inhibits apoptosis
  • present with painless “waxing and waning” lymphadenopathy
53
Q

adult T cell lymphoma

A
  • occurs in adults
  • caused by HTLV-1 (associated with IV drug abuse)
  • adults present with cutaneous lesions, esp affects populations in Japan, West Africa, and the Carribean
  • Lytic bone lesions, hypercalcemia
54
Q

mycosis fungoides/Sezary syndrome

A
  • common in adults
  • adults present with cutaneous patches/plaques/tumors with potential to spread to lymph nodes and viscera
  • circulating malignant cells seen in Sezary syndrome
  • indolent, CD4+
55
Q

ALL

A
  • age < 15 years
  • T cell ALL can present with mediastinal mass (dysphagia, dyspnea)
  • associated with Downs “we ALL fall down”
  • TdT +
  • B cell form is CD10+
  • most responsive to therapy
  • may spread to the CNS and testes
  • t( 12;21) has better prognosis
56
Q

Small lymphocytic lymphoma/Chronic lymphocytic leukemia

A
  • age > 60 years
  • CD 20+, CD5+ B cell neoplasm
  • often asymptomatic, progresses slowly, smudge cells in peripheral blood smear, autoimmune hemolytic anemia
  • SLL is the same as CLL except CLL has increased peripheral blood lymphocytosis or BM involvement
57
Q

hairy cell leukemia

A
  • age: adults, mature B cell tumor of the elderly
  • cells have filamentous, hair-like projections
  • TRAP +ve
  • causes marrow fibrosis (dry tap)
  • treat wtih cladribine (2CDA), an adenosine analog (inhibits adenosine deaminase)
58
Q

AML

A
  • median onset 65 years
  • Auer rods, peroxidase +, cytoplasmic inclusions seen in M3
  • risk factors: prior exposure to alkylating chemo, radiation, myeloproliferative disorders, downs
  • t(15;17) –> M3 responds to all-trans retinoic acid
  • M3 due to PML/RARa fusion gene which prevents differentation
  • can present with DIC if Aurer rods are released into plasma
59
Q

CML

A
  • peak incidence 45-85 years
  • defined by philadelphia chromosome t(9;22)
  • very low leukocyte alk phos
  • responds to imatinib (tyrosine kinase inhibitor)
60
Q

9;22 translocation

A
  • philadelphia chromosome - CML
61
Q

8;14 translocation

A

Burkitt lymphoma (c-myc activation)

62
Q

11;14 translocation

A

mantle cell lymphoma (cyclin D1 activation)

63
Q

14;18

A

follicular lymphoma (bcl2 activation)

64
Q

15;17

A

M3 type AML

65
Q

Langerhans cell histiocytosis

A
  • lytic bone lesions and skin rash or recurrent OM with mastoid mass
  • immature APCs
  • express S-100 and CD1a
  • have Birbeck “tennis racket” granules
66
Q

Polycythemia Vera

A
  • a chronic myeloproliferative disorder where hct > 55%
  • somatic (non-hereditary) mutation of Jak2 gene – receptor associated tyrosine kinase
  • often presents as intense itching after the shower
  • rare but classic symptom is erythromelalgia (severe, burning pain and reddish or bluish discoloration) due to episodic blood clots in extremity vessels
67
Q

essential thrombocytosis

A
  • similar to polycythemia vera, but specific overproduction of abnormal platelets –> bleeding, thrombosis
  • bone marrow contains enlarged megakaryocytes
68
Q

myelofibrosis

A
  • fibrotic obliteration of the BM

- teardrop RBCs and immature forms of the myeloid line

Step 1 Flash Cards (36 decks)

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