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Compiled PPS board reviewer > 3 hema-onco > Flashcards

3 hema-onco Flashcards

1
Q

low Hgb

low MCV

A

IDA
Thalassemia
Sideroblastic Anemia
Anemia of Chronic dse

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2
Q

low Hgb
Normal MCV
retic ct <3%

A 
infection
drugs
lead poisoning
acute blood loss
anemia of chronic dse
renal dse
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3
Q

low hgb

high MCV

A

drugs
vitamin B12/ folate
immune hemolytic
diamond-blackfan

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4
Q

low hgb
normal MCV
retic >3%
with signs of hemolysis

A 
hereditary spherocytosis
hereditary elliptocytosis
G6PD
sickle cell dse
HUS/TTP
Mechanical Heart Valve
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5
Q

MC nutritional deficiency in children

A

IDA

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6
Q

Risk factors of IDA in infants and toddlers

A 
LBW
prematurity 
perinatal blood loss
early cord clamping
excessive consumption of cows milk
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7
Q

risk factor of IDA in children and adolescents

A

inc reqt (growth spurt and pregn ancy)
occult/chronic blood loss (peptic ulcer, polyp)
menstrual blood loss
infection with intestinal hookworm, trichuris, plasmodium, helicobater pylori, giardia lamblia

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8
Q

most important clinical sign of IDA

A

pallor

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9
Q

manifestation of anemia @ hgb 6-10g/dL

A

mild irritability

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10
Q

manifestation of IDA @ hgb 7-8g/dL

A

pallor

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11
Q

manifestation of IDA @ hgb <5g/dL

A 
lethargy, 
anorexia, 
easy fatigability, 
systolic flow murmur,
high output cardiac failure
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12
Q

koilonychia:
pica:
pagophagia:
plumbism:

A

koilonychia: spoon nails
pica: desire to eat non-nutritive substance
pagophagia: desire to ingest ice
plumbism: ingest lead

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13
Q

PBS of IDA

A

microcytic hypochromic RBC (d/t dec Hgb production or faulty function)

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14
Q

response of iron therapy in 12-24hrs

A

subjective improvement (increase appetite, decrease irritability

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15
Q

response of iron therapy in 36-48hrs

A

initial bone marrow response (erythroid hyperplasia)

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16
Q

response of iron therapy in 48-72hrs

A

reticulocystosis w/c peak at 5-7 days

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17
Q

response of iron therapy in 4-30 days

A

inc hgb level

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18
Q

response of iron therapy in 1-3 months

A

repletion of iron stores

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19
Q

treatment of IDA

A

Elemental iron 4-6mg/kg TID x 8 weeks

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20
Q

hallmark of aplastic anemia

A

peripheral pancytopenia with marrow hypoplasia or aplasia

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21
Q

moderate aplastic anemia
ANC:
PC:
corrected retic ct:

A

ANC: 500-1000/mm3
PC: 20K-100K/mm3
retic ct: <1%

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22
Q

severe aplastic anemia
ANC:
PC:
corrected retic ct:

A

ANC: <500
PC: <20K
retic ct: <1%

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23
Q

treatment of choice for aplastic anemia

A

allogenic hematopoietic stem cell transplant

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24
Q

TOC for aplastic anemia if w/o HLA-matched sibling or donor

A

immunosuppresive therapy with horse anti-thymocyte globulin and cyclosporine

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25
Q

MC inherited aplastic anemia

A

Fanconi Anemia

  • AR
  • 3-14 yo
  • cells cant properly repair DNA damage known as interstrand crosslink (abnormal chromosomal fragility)
  • inability of fanconi cell to remove O2 free radicals
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26
Q

diagnostic criteria of Shwachman-Diamond syndrome

A

exocrine pancreatic insufficiency & variable hematologic cytopenia d/t bone marrow failure

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27
Q

Criteria of Diamond Blackfan Syndrome

A

<1yo
macrocytic anemia w/ no other cytopenia
reticulocytopenia
normal marrow cellularity w/a paucity of erythroid precursors

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28
Q

mainstay of therapy for Diamond Blackfan Syndrome

A

steroids

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29
Q

treatment of shwachman diamond syndrom

A

oral pancreatic enzyme replacement
fat soluble vitamins
BT, G-CSF
hematppoietic stem cell transplant

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30
Q

MC inherited enzymatic disorder

A

Glucose-6-Phosphate Dehydrogenase Deficiency

  • x linked
  • most important dse of HMP pathway
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31
Q

clinical syndrome of G6PD

A
  • episodic hemolytic anemia induced by infection, drugs, fava beans
  • spontaneous chronic non-spherocytic hemolytic anemia
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32
Q

PBS of G6PD deficiency

A

low hgb
heinz bodies
anisopoikilocytosis
bite cells

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33
Q

Manifestation of Alpha Thalassemia

A

Hydrops Fetalis w/ Barts Hgb: SEVERE MICROCYTIC anemia
Thalassemia Major: MOD MICROCYTIC anemia
Thalassemia Minor: MILD MICROCYTIC anemia
Silent Carrier: Normal hgb and MCV

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34
Q

Manifestation of Beta Thalassemia

A

B thalassemia Major: SEVERE MICROCYTIC anemia with TARGET CELLS; transfusion dependent
B thalassemia Intermedia: MOD MICROCYTIC anemia; nontransfusion dependent
B thalassemia Minor/trait: MILD MIRCOCYTIC anemia

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35
Q

PBS of thalassemia

A

microcytic hypochromic RBC
target cell
Heinz bodies

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36
Q

MC cause of hemolytic anemia d/t red cell membrane defect

A

Hereditary Spherocytosis

- d/t abnormalities of ankyrin & spectrin (proteins involved in RBC cytoskeleton)

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37
Q

confirms presence of fragile sphere shaped RBCs

A

osmotic fragility test

38
Q

PBS of hereditary spherocytosis

A

spherocytes

39
Q

clinical hallmark of sickle cell dse

A

vasoocclusive phenomena & hemolysis

40
Q

skull radiograph of sickle cell dse

A

crew cut or hair on end appearance

41
Q

PBS of sickle cell dse

A

Howell Jolly Bodies & sickle cell

42
Q

preferred diagnosis for sickle cell dse

A

high performance liquid chromatography

43
Q

MC acquired red cell aplasia

A

Transient Erythroblastopenia of childhood

  • 6mos to 3yo
  • decreased reticulocytes and bone marrow erythroid precursor
  • normal MCV
  • recovers in 1-2 mos
  • transfuse as needed
44
Q

best documented viral cause of RBC aplasia assoc w/ chronic hemolysis

A

parvo B19

Red cell aplasia assoc w/ chronic hemolysis
- recovery <2wks in normal children
- aplastic crisis in patients w/ hemolysis:
» erythroid marrow failure
» reticulocytopenia
» rapid dec in Hgb & Hct

45
Q

macrocytic anemia
neutropenia
thrombocytopenia
marrow with ringed sideroblasts

A

Pearson Marrow - Pancreas Syndrome

  • failure to thrive
  • marrow failure in neonatal period
  • exocrine pancreas deficiency
  • muscle and neurologic impairment
  • early death
46
Q

absolute retic ct of Anemia of Chronic Dse

A

normal to low

PBS: normochromic normocytic

47
Q

treatment for anemia of renal dse

A

EPO & iron

  • mild hemolysis, dec EPO production d/t damaged renal cells
48
Q

duration of physiologic anemia of infancy

A

within 1st week of life, progressive decline in hgb level begin & persist for 6-8wks (2-4mos)

**stored iron is sufficient for hgb synthesis until 20wks of age (5mos)

49
Q

spontaneous resolution of physiologic anemia in prematurity

A

40wks AOG

physiologic anemia of prematurity

  • hgb of 7-9g/dL are reached by 3-6wks
  • short survival of RBC (40-60days)
  • early preterm transfused RBC is 30days
50
Q

ineffective erythropoiesis: premature cell death, decrease output of RBC from marrow leading to anemia

A

Megaloblastic anemia

  • RBC are large (inc MCV) & oval
  • hypersegmented neutrophils having >5 lobes
  • result from deficiency of folic acid and vitamin B12
  • occurs after 2-3mos of folate free diet
51
Q

normal infant daily requirement of folic acid

A

25-35 ug/day

52
Q

peak incidence of megaloblastic anemia 2 to folate deficiency

A

4-7 months

53
Q

causes of folate deficiency

A

malabsorption and chronic diarrhea
phenytoin, phenobarbital, primidone
methotrexate, pyrimithamine, trimethoprim

54
Q

diagnostics for megaloblastic anemia 2 to folate deficiency

A

PBS: macrocytic, low retic ct, nucleated RBC, large neutrophils
serum folic acid <3 ng/mL
RBC folate: better indicator of chronic deficiency
(NV: 150-600ng/ml of packed cells)
LDH markedly elevated
hypercellular BM: erythroid hyperplasia

55
Q

conditions leading to impaired Vit B12 absorption

A 
NEC
regional enteritits
terminal ileum removed
Imerslund-Grasbeck syndrome
absence of transport CHON transcobalamin
56
Q

diagnostic for megaloblastic anemia 2 to Cobalamin deficiency

A

PBS: macrocytic, macroovalocytosis
elevated methylmalonic acid & homocysteine
excessive excretion of methylmalonic acid in urine (nv: 0-3.5mg/24hrs); reliable & sensitive index

57
Q

treatment for neurologic symptoms of vitamin B12 deficiency

A

vit B12 1mg IM OD x 2wks

58
Q

acquired and hereditary d/o of heme synthesis

A

sideroblastic anemia

  • hypochromic, microcytic
  • inc RDW, serum iron and transferrin
  • retention of iron in mitochondria: ringed sideroblasts
59
Q

major complication of sideroblastic anemia

A

iron overload

60
Q

mc molecular defect of of hereditary spherocytosis

A

spectrin or ankyrin

61
Q

age of functional asplenia

A

6mos-5yo

62
Q

MC glycolytic enzyme defect as a cause of hemolytic anemia

A

pyruvate kinase deficiency

63
Q

hallmark of autoimmune hemolytic anemia

A

(+) direct coombs test

64
Q

Diagnostic criteria for polycythemia rubra vera

A 
3M or 1M2mm
MAJOR:
- inc RBC mass
- o2 sat 92%
- palpable splenomegaly
MINOR:
- PC >400
- leukocytosis >12
- inc leukocyte alkaline phosphatase
- inc vit B12 >900mg/ml
65
Q

MC and serious congenital coagulation factor deficiency

A

Hemophilia A or B

  • delayed clotting of blood caused by deficiency of clotting factors
  • easy bruising, hematoma, hemarthroses when child begin to cruise
66
Q

hallmark of hemophilia

A

hemarthrosis

67
Q

factor VIII deficiency

A

hemophilia A

68
Q

factor IX deficiency

A

hemophilia B

69
Q

factor XI deficiency

A

hemophilia C

70
Q

most common earliest joint involved

A

ankle

71
Q

hemophilia A mgt alternative

A

cryoprecipitate
FFP
emicizumab

72
Q

hemophilia B & C mgt alternative

A

cryoprecipitate

FFP

73
Q

MC inherited bleeding Disorder

A

Von Willebrand Disease

  • disturbs both primary & secondary hemostasis
  • inc bleeding time causing mucous membrane bleeding, petechiae, purpura
  • often has a family hx of bleeding
74
Q

Diagnostics of vWD

A
  • dec vWF
  • dec Factor 8
  • prolong bleeding time
  • abnormal platelet adhesion
  • inc PTT
  • Ristocetin cofactor assay (measures vWF antigen level & activity)
75
Q

Treatment for vWF

A
  • Desmopressin 0.3ug/kg (inc amt of circulating vWF by releasing from storage
  • replacement therapy
  • antifibrinolytics
  • hormonal treatment w/ estrogen in woman
76
Q

Major symptom of vWD

A

menorrhagia

77
Q

MC cause of acute onset thrombocytopenia in children

A

Idiopathic Thrombocytopenic Purpura –> immune thrombocytopenia

  • 14 wks after viral infection
  • spontaneous resolution in 6 months
78
Q

treatment for ITP

A

IVIG 0.8-1 g/kg/day for 1-2 days
Prednisone 1-4MKD for 2-3 wks
IV anti D therapy

79
Q

Drugs that induce thrombocytopenia

A

valproic acid
phenytoin
sulfonamide
heparin

80
Q

Pentad of Thrombotic Thrombocytic Purpura

A 
Fever
Anemia (microangiopathic hemolytic anemia)
Thrombocytopenia
Renal dysfunction
Nervous system changes
81
Q

Diagnostics of TTP

A

schistocytes, spherocytes, helmet cells
elevated retic ct
thrombocytopenia
elevated BUN, CREA

82
Q

treatment of TTP

A

plasmapheresis
Rituximab
steroids
splenctomy

83
Q

triad of Kassabach Merrit Syndrome

A

cavernous hemangioma
severe thrombocytopenia
intravascular coagulation

giant hemangioma with localized intravascular coagulation –>
decrease platelet and hypofibrinogenemia

84
Q

triad of Wiskott Aldrich Syndrome

A

Eczema
thrombocytopenic hemorrhage
immunologic defect

normal number of megakaryocytes but abnormal morphology
small sized platelets
lymphoreticular malignancies

85
Q

autosomal recessive inherited platelet adhesion defect d/t GP Ib deficiency

A

Bernard Soulier Syndrome

  • moderate thrombocytopenia, large platelet & decreased
  • risocetin induced aggregation not corrected by addition of vWF
86
Q

AR, deficiency of platelet fibrinogen receptor GPIIb-IIIa

A

Glanzmann Thrombastenia

- normal plt ct, absent aggregation in vitro, absent clot retraction

87
Q

rare AR disorder w/c there is an absence of fibrinogen

A

congenital afibrinogenemia

  • do not bleed as frequent as patients w/ hemophilia
  • prolonged PT, PTT & thrombin time
88
Q

dysfunctional fibrinogens

A

dysfibrinogenemia

tx: FFP, cryoprecipitate

89
Q

MC malignant neoplasm in <15yo

A

leukemia

90
Q

peak age of ALL

A

2-6Y/O

91
Q

most important morphologic feature of ALL

A

FAB L3 subtype

92
Q

manifestation of ALL

A
  • anorexia, fatigue, irritability, intermittent low grade fever, bone & joint pains (lower extremities)
  • history of URTI 1-2months prior
  • pallor, bruising, epistaxis

Compiled PPS board reviewer (60 decks)

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