Heme Onc Flashcards

(68 cards)

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
orotic aciduria
- 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
26
intravascular hemolysis
- decreased haptoglobin, increased LDH, schistocytes and reticulocytes, urobilinogen in urine
27
extravascular hemolysis
- spherocytes, increased LDH + increased unconjugated bili
28
anemia of chronic disease
- 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
G6PD
- 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
pyruvate kinase deficiency
- AR defect in pyruvate kinase - decreased ATP and rigid RBCs - hemolytic anemia of the newborn
31
Hgb C defect
- 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
PNH
- 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
Warm agglutinin hemolytic anemia
- IgG mediated, can be seen with SLE, CLL and certain drugs (penicillin, methyldopa) - coombs +ve
34
cold agglutinin hemolytic anemia
- IgM mediated, acute anemia triggered by cold, seen in CLL, mycoplasma pneumonia, and infectious mono - coombs +ve
35
lead poisoning | - enzymes affected, accumulated substrate and presenting symptoms
- ALA synthase and ferrochelatase - protoporphyrin, d-ALA - microcytic anemia, GI and kidney disease
36
acute intermittent porphyria | - enzyme affected, accumulated substrate and presenting symptoms
- 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
porphyria cutanea tarda | - enzyme affected, accumulated substrate and presenting symptoms
- uroporphyrinogen decarboxylase - uroporphyrin (tea colored urine) - blistering cutaneous photosensitivity - most common porphyria
38
Bernard-Soulier syndrome
- dec platelet count, increased bleeding time | - defect in platelet plug formation due to decreased Gp1b --> defect in platelet-vWF adhesion
39
Glanzmann thrombasthenia
- increased bleeding time - defect in platelet plug formaiton due to decreased GpIIb/IIIa --> defective platelet aggregation - blood smear shows no platelet clumping
40
immune thrombocytopenia
- 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
TTP
- 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
vWF disease
- 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
Factor V Leiden
- 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
prothrombin gene mutation
- mutation in the 3' untranslated region --> increased production of prothrombin --> increased plasma levels and venous clots
45
antithrombin deficiency
- antithrombin deficiency - doesnt have any direct effect on PT/PTT, but PTT will not increase as much with heparin administration
46
protein C or S deficiency
- decreased ability to inactivate factors V and VIII | - increased risk of thrombotic skin necrosis with hemorrhage following coumadin
47
leukemoid reaction vs. CLL
- leukemoid reaction will have increased ALP | - CLL will have decreased ALP
48
Reed-Sternberg cells
- 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
Burkitt Lymphoma
- 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
diffuse large B cell lymphoma
- usually older adults, 20% kids - t (14;18) - most common type of non-Hodgkin lymphoma in adults
51
mantle cell lymphoma
- older males - t (11;14) translocation of cyclin D1 and heavy chain IgG - CD5+
52
follicular cell lymphoma
- 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
adult T cell lymphoma
- 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
mycosis fungoides/Sezary syndrome
- 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
ALL
- 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
Small lymphocytic lymphoma/Chronic lymphocytic leukemia
- 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
hairy cell leukemia
- 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
AML
- 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
CML
- peak incidence 45-85 years - defined by philadelphia chromosome t(9;22) - very low leukocyte alk phos - responds to imatinib (tyrosine kinase inhibitor)
60
9;22 translocation
- philadelphia chromosome - CML
61
8;14 translocation
Burkitt lymphoma (c-myc activation)
62
11;14 translocation
mantle cell lymphoma (cyclin D1 activation)
63
14;18
follicular lymphoma (bcl2 activation)
64
15;17
M3 type AML
65
Langerhans cell histiocytosis
- 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
Polycythemia Vera
- 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
essential thrombocytosis
- similar to polycythemia vera, but specific overproduction of abnormal platelets --> bleeding, thrombosis - bone marrow contains enlarged megakaryocytes
68
myelofibrosis
- fibrotic obliteration of the BM | - teardrop RBCs and immature forms of the myeloid line