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EOR Family med > Heme > Flashcards

Heme Flashcards

(78 cards)

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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
dx hemophilia A
low factor 8 prolonged aPTT, normal PT, platelet levels PTT corrects with mixing studies
26
tx hemophilia A
factor 8 infusion desmopressin
27
dx hemophilia B
low factor 9 prolonged aPTT, normal PT and platelets prolonged PTT corrects w mixing studies
28
tx hemophilia B
factor 9 infusion desmopressin not useful
29
MC inherited type of hyper coagulability (thrombophilia)
Factor 5 leiden mutation (activated protein C resistance)
30
Pathophysiology factor 5 leiden
mutated factor 5 is resistant to breakdown by activated protein C
31
sx factor 5 leiden
increased incidence of DVT, PE, hepatic vain or cerebral vein thrombosis increased risk of miscarriage, preeclampsia, placental abruption, stillbirth
32
is factor 5 leiden associated w increased risk of MI or CVA
no
33
dx factor 5 leiden mutation
activated protein C resistance assay --> if positive, confirm w DNA testing normal PT and PTT
34
tx factor 5 leiden mutation
asx - no anticoagulation severe - indefinite anticoagulation (warfare in direct oral anticoagulant)
35
Pathophysiology of protein C or S deficiency
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
sx protein C or S deficiency
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
tx protein C or S deficiency
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
polycythemia vera
overproduction of all 3 hematopoietic myeloid stem cell lines
39
polycythemia vera is caused by what mutation
JAK2
40
sx polycythemia vera
pruritus esp after a hot shower or bath epistaxis bleeding DVT, TIA hepatosplenomegaly facial plethora (flushing) engorged retinal veins
41
MC childhood malignancy
acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL) peaks 2-5 years
42
sx ALL/LBL
pancytopenia - fever and infections, bleeding, hepatomegaly or splenogemaly lymphadenopathy
43
dx ALL/LBL
WBC 5000-100,000, anemia, thrombocytopenia bone marrow aspiration: hyper cellular w > 20% blasts (definitive)
44
MC form of leukemia in adults
chronic lymphocytic leukemia/small lymphocytic lymphoma
45
Pathophysiology chronic lymphocytic leukemia/small lymphocytic lymphoma
clonal proliferation of morphologically mature but immunologically and functionally incompetent B cells
46
sx chronic lymphocytic leukemia/small lymphocytic lymphoma
usually asx pancytopenia - fatigue, anemia, increased infections (neutropenia), bleeding lymphadenopathy**** splenomegaly
47
dx chronic lymphocytic leukemia/small lymphocytic lymphoma
lymphocytosis absolute lymphocytosis >/= 5,000/mcL - small, well differentiated, normal and mature-appearing lymphocytes scattered "smudge cells" hypogammaglobulinemia (decreased IgG, IgA, IgM)
48
MC acute leukemia in adults
acute myeloid leukemia
49
dx acute myeloid leukemia
normocytic normochromic anemia w normal or decreased reticulocytes circulating myeloblasts >/= 20% myeloblasts auer rods (pink/red rod-like granular structures in the cytoplasm)
50
Pathophysiology of chronic myelogenous leukemia
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
sx chronic myelogenous leukemia
pruritus after hot bath/shower splenomegaly
52
dx chronic myelogenous leukemia
leukocytosis with granulocytic cells (basophilia, neutrophilic, and eosinophilia) decreased leukocyte alkaline phosphatase score fluorescence in situ hybridization (FISH) - genetic testing for Philadelphia chromosome
53
hodgkin lymphoma
B cell malignancy
54
ages commonly affected Hodgkin lymphoma
peaks at 20 years and then again > 50
55
RF hodgkin lymphoma
epstein-barr virus immunosuppression smoking Caucasian
56
MC type of Hodgkin lymphoma
nodular sclerosing - female predominance
57
which type of Hodgkin lymphoma is associated w Epstein Barr virus
mixed cellularity
58
which type of Hodgkin lymphoma has the best prognosis
lymphocyte rich/predominant
59
which type of Hodgkin lymphoma has the worst prognosis
lymphocyte depleted
60
sx hodgkin lymphoma
painless lymphadenopathy - ETOH ingestion may induce lymph node pain (cervical and supraclavicular) hepatomegaly, splenomegaly night sweats, weight loss, Pet-Ebstein fever (cyclical fever)
61
dx hodgkin lymphoma
excision whole lymph node biopsy - Reed-Sternberg cells - large cells w bi or multilines nuclei (owl eye appearance) and inclusions in nucleoli
62
infections associated w non-hodgkin lymphoma
epstein barr HIV HHV 8 H pylori
63
thrombotic thrombocytopenic purpura is due to
ADAMTS 13 deficiency --> leads to large vWF multimers that cause small vessel thrombosis
64
sx TTP
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
dx TTP
thrombocytopenia w normal coagulation studies
66
tx TTP
plasma exchange glucocorticoids and/or Rituximab If no response
67
RF hemolytic uremic syndrome (HUS)
predominantly seen in kids < 5 with a recent hx of gastroenteritis
68
sx HUS
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
dx HUS
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
tx HUS
supportive if severe - plasmapheresis
71
what agents are avoided in tx of HUS
antibiotics and anti-motility agents bc they may worsen the condition
72
causes of DIC
infections - bacterial sepsis MC malignancies OB - pre-eclampsia massive tissue injury and trauma
73
sx DIC
oozing from venipuncture sites, catheters, drains, extensive bruising, bleeding from multiple sites thrombosis - gangrene or multi-organ dysfunction
74
dx DIC
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
Pathophysiology immune thrombocytopenic purpura
autoantibodies against platelets, leading to splenic destruction of platelets
76
sx ITP
mucocuteanous bleeding not associated w splenomegaly****
77
dx ITP
isolated thrombocytopenia normal coagulation studies bone marrow: megakaryocytes (large sized platelets)
78
tx ITP
glucocorticoids or IVIG or anti-D Rituximab or TPO receptor agonist or splenectomy if refractory

EOR Family med (25 decks)

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