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EOR Family med > Heme > Flashcards

Heme Flashcards

1
Q

inheritance in G6PD

A

x linked recessive enzymatic disorder

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2
Q

sx G6PD

A

asx until times of oxidative stress
episodic hemolytic anemia - sx begin 2-4 days after exposure of precipitants - fatigue, pallor, jaundice, dark urine, back or ab pain

may have neonatal jaundice

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3
Q

peripheral smear G6PD

A

normocytic hemolytic anemia only during crises - schistocytes (bite or fragmented cells) + Heinz bodies are hallmark

smear normal when not in acute stage

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4
Q

Most sensitive test for G6PD

A

rapid fluorescent spot test - positive if blood spot fails to fluoresce under UV light

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5
Q

Dx IDA

A

microcytic (MCV) hypochromic (MCHC) anemia
increased RDW
Decreased ferritin < 30
increased TIBC
decreased transferrin saturation (< 20-15%)
decreased serum iron

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6
Q

normal MCHC values

A

32-36

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7
Q

peripheral smear lead poisoning anemia (plumbism)

A

microcytic hypochromic anemia w basophilic stippling
ringed sideroblasts on bone marrow

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8
Q

radiographs for plumbism

A

lead lines - linear hyperdensities at the metaphysical plates in kids

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9
Q

when should you think about thalassemia

A

microcytic anemia
increased iron or no response to iron replacement therapy

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10
Q

MC cause B12 anemia

A

pernicious anemia

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11
Q

what type of IBD is more susceptible to B12 deficiency

A

Crohn disease

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12
Q

Dx B12 deficiency

A

increased MCV + megaloblastic anemia (macro-ovalocytes and hyperhsegmented neutrophils w > 5 lobes)

decreased serum B12

increased homocysteine
increased methylmalonic acid (MMA)

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13
Q

Dx folate deficiency

A

Increased MCV + megaloblastic anemia (hyperhsegmented neutrophils, macro-ovalocytes)
decreased serum folate
increased homocysteine
normal methylmalonic acid (MMA)

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14
Q

dx hereditary spherocytosis

A

hyper chromic microcytosis
spherocytes
increased MCHC
negative Coombs test

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15
Q

tx hereditary spherocytosis

A

folic acid
splenectomy is curative

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16
Q

PTT measures

A

1, 2, 5, 8, 9, 11, 12

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17
Q

PT measures

A

1, 2, 5, 7, 10

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18
Q

MC hereditary bleeding disorder

A

Von Willebrand disease

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19
Q

sx Von Willebrand dz

A

prolonged bleeding (oral, uterine, GI)

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20
Q

coagulation studies for von willebrand

A

normal or prolonged PTT that corrects with mixing study
PT is not affected
PTT and bleeding time prolonged and worse w aspirin
platelet count usually normal

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21
Q

in what subtype of von willebrands are platelets decreased

A

type 2B

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22
Q

what other factor may be decreased in von willebrand

A

factor 8

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23
Q

tx von willebrand

A

Desmopressin
IV Von Willebrand factor with factor 8 (severe)

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24
Q

Inheritance of hemophilia A and B

A

X linked recessive

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25
Q

dx hemophilia A

A

low factor 8
prolonged aPTT, normal PT, platelet levels
PTT corrects with mixing studies

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26
Q

tx hemophilia A

A

factor 8 infusion
desmopressin

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27
Q

dx hemophilia B

A

low factor 9
prolonged aPTT, normal PT and platelets
prolonged PTT corrects w mixing studies

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28
Q

tx hemophilia B

A

factor 9 infusion
desmopressin not useful

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29
Q

MC inherited type of hyper coagulability (thrombophilia)

A

Factor 5 leiden mutation (activated protein C resistance)

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30
Q

Pathophysiology factor 5 leiden

A

mutated factor 5 is resistant to breakdown by activated protein C

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31
Q

sx factor 5 leiden

A

increased incidence of DVT, PE, hepatic vain or cerebral vein thrombosis
increased risk of miscarriage, preeclampsia, placental abruption, stillbirth

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32
Q

is factor 5 leiden associated w increased risk of MI or CVA

A

no

33
Q

dx factor 5 leiden mutation

A

activated protein C resistance assay –> if positive, confirm w DNA testing
normal PT and PTT

34
Q

tx factor 5 leiden mutation

A

asx - no anticoagulation
severe - indefinite anticoagulation (warfare in direct oral anticoagulant)

35
Q

Pathophysiology of protein C or S deficiency

A

proteins C and S are vitamin K dependent anticoagulant proteins produced by the liver that stimulate fibrinolysis and inactivate factors five and 8

decreased protein C or S leads to hyper coagulability

36
Q

sx protein C or S deficiency

A

increased incidence of DVT and PE
Warfarin-induced skin necrosis (and pt presenting w this needs to be tested)
purpura fulminan in newborns - red purpuric lesions at pressure points, progresses to painful black eschars

37
Q

tx protein C or S deficiency

A

protein C concentrate. indefinite anticoagulation (warfarin or direct oral anticoagulation)

warfarin induced necrosis - D/C, administer IV vitamin K, therapeutic heparin, protein C concentrate or fresh frozen plasma.

38
Q

polycythemia vera

A

overproduction of all 3 hematopoietic myeloid stem cell lines

39
Q

polycythemia vera is caused by what mutation

A

JAK2

40
Q

sx polycythemia vera

A

pruritus esp after a hot shower or bath
epistaxis
bleeding
DVT, TIA
hepatosplenomegaly
facial plethora (flushing)
engorged retinal veins

41
Q

MC childhood malignancy

A

acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL)

peaks 2-5 years

42
Q

sx ALL/LBL

A

pancytopenia - fever and infections, bleeding, hepatomegaly or splenogemaly
lymphadenopathy

43
Q

dx ALL/LBL

A

WBC 5000-100,000, anemia, thrombocytopenia
bone marrow aspiration: hyper cellular w > 20% blasts (definitive)

44
Q

MC form of leukemia in adults

A

chronic lymphocytic leukemia/small lymphocytic lymphoma

45
Q

Pathophysiology chronic lymphocytic leukemia/small lymphocytic lymphoma

A

clonal proliferation of morphologically mature but immunologically and functionally incompetent B cells

46
Q

sx chronic lymphocytic leukemia/small lymphocytic lymphoma

A

usually asx
pancytopenia - fatigue, anemia, increased infections (neutropenia), bleeding
lymphadenopathy**
splenomegaly

47
Q

dx chronic lymphocytic leukemia/small lymphocytic lymphoma

A

lymphocytosis
absolute lymphocytosis >/= 5,000/mcL - small, well differentiated, normal and mature-appearing lymphocytes
scattered “smudge cells”
hypogammaglobulinemia (decreased IgG, IgA, IgM)

48
Q

MC acute leukemia in adults

A

acute myeloid leukemia

49
Q

dx acute myeloid leukemia

A

normocytic normochromic anemia w normal or decreased reticulocytes
circulating myeloblasts
>/= 20% myeloblasts
auer rods (pink/red rod-like granular structures in the cytoplasm)

50
Q

Pathophysiology of chronic myelogenous leukemia

A

uncontrolled production of mature and maturing granulocytes (predominantly neutrophils)

fusion of BCR (on chromosome 22) and ABL1(on chromosome 9) = BCR-ABL2 fusion

translocation btwn chromosomes 9 and 22 = Philadelphia chromosome

51
Q

sx chronic myelogenous leukemia

A

pruritus after hot bath/shower
splenomegaly

52
Q

dx chronic myelogenous leukemia

A

leukocytosis with granulocytic cells (basophilia, neutrophilic, and eosinophilia)

decreased leukocyte alkaline phosphatase score

fluorescence in situ hybridization (FISH) - genetic testing for Philadelphia chromosome

53
Q

hodgkin lymphoma

A

B cell malignancy

54
Q

ages commonly affected Hodgkin lymphoma

A

peaks at 20 years and then again > 50

55
Q

RF hodgkin lymphoma

A

epstein-barr virus
immunosuppression
smoking
Caucasian

56
Q

MC type of Hodgkin lymphoma

A

nodular sclerosing - female predominance

57
Q

which type of Hodgkin lymphoma is associated w Epstein Barr virus

A

mixed cellularity

58
Q

which type of Hodgkin lymphoma has the best prognosis

A

lymphocyte rich/predominant

59
Q

which type of Hodgkin lymphoma has the worst prognosis

A

lymphocyte depleted

60
Q

sx hodgkin lymphoma

A

painless lymphadenopathy - ETOH ingestion may induce lymph node pain (cervical and supraclavicular)
hepatomegaly, splenomegaly
night sweats, weight loss, Pet-Ebstein fever (cyclical fever)

61
Q

dx hodgkin lymphoma

A

excision whole lymph node biopsy - Reed-Sternberg cells - large cells w bi or multilines nuclei (owl eye appearance) and inclusions in nucleoli

62
Q

infections associated w non-hodgkin lymphoma

A

epstein barr
HIV
HHV 8
H pylori

63
Q

thrombotic thrombocytopenic purpura is due to

A

ADAMTS 13 deficiency –> leads to large vWF multimers that cause small vessel thrombosis

64
Q

sx TTP

A

thrombocytopenia - mucocutaneous bleeding (epistaxis, bleeding gums, petechiae, purpura)

microangiopathic hemolytic anemia - anemia, jaundice, schistocytes, splenomegaly

neuro sx - HA, visual changes, confusion, somnolence, seizures

Fever (rare)

Kidney dysfunction - AKI uncommon

65
Q

dx TTP

A

thrombocytopenia w normal coagulation studies

66
Q

tx TTP

A

plasma exchange
glucocorticoids and/or Rituximab If no response

67
Q

RF hemolytic uremic syndrome (HUS)

A

predominantly seen in kids < 5 with a recent hx of gastroenteritis

68
Q

sx HUS

A

prodromal diarrheal illness (bloody, N/V) 5-10 days before renal manifestations (oliguria, hematuria)

thrombocytopenia (bleeding)

microangiopathic hemolytic anemia - splenomegaly

renal dysfunction - uremia (elevated BUN)

69
Q

dx HUS

A

thrombocytopenia with normal coagulation studies (PT, PTT, fibrinogen)

hemolysis - schistocytes etc

Increased BUN and creatinine

positive stool culture or detectable antibody to Shiga toxin

70
Q

tx HUS

A

supportive
if severe - plasmapheresis

71
Q

what agents are avoided in tx of HUS

A

antibiotics and anti-motility agents bc they may worsen the condition

72
Q

causes of DIC

A

infections - bacterial sepsis MC
malignancies
OB - pre-eclampsia
massive tissue injury and trauma

73
Q

sx DIC

A

oozing from venipuncture sites, catheters, drains, extensive bruising, bleeding from multiple sites
thrombosis - gangrene or multi-organ dysfunction

74
Q

dx DIC

A

decreased fibrinogen (widespread activation of clotting cascade)
prolonged PT and activated PTT
increased INR
elevated fibrin and D dimer

peripheral smear - thrombocytopenia, fragmented RBCs, schistocytes

75
Q

Pathophysiology immune thrombocytopenic purpura

A

autoantibodies against platelets, leading to splenic destruction of platelets

76
Q

sx ITP

A

mucocuteanous bleeding
not associated w splenomegaly**

77
Q

dx ITP

A

isolated thrombocytopenia
normal coagulation studies

bone marrow: megakaryocytes (large sized platelets)

78
Q

tx ITP

A

glucocorticoids or IVIG or anti-D

Rituximab or TPO receptor agonist or splenectomy if refractory

EOR Family med (25 decks)

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