3 hema-onco Flashcards
1
Q
low Hgb
low MCV
A
IDA
Thalassemia
Sideroblastic Anemia
Anemia of Chronic dse
2
Q
low Hgb
Normal MCV
retic ct <3%
A
infection drugs lead poisoning acute blood loss anemia of chronic dse renal dse
3
Q
low hgb
high MCV
A
drugs
vitamin B12/ folate
immune hemolytic
diamond-blackfan
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
5
Q
MC nutritional deficiency in children
A
IDA
6
Q
Risk factors of IDA in infants and toddlers
A
LBW prematurity perinatal blood loss early cord clamping excessive consumption of cows milk
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
8
Q
most important clinical sign of IDA
A
pallor
9
Q
manifestation of anemia @ hgb 6-10g/dL
A
mild irritability
10
Q
manifestation of IDA @ hgb 7-8g/dL
A
pallor
11
Q
manifestation of IDA @ hgb <5g/dL
A
lethargy, anorexia, easy fatigability, systolic flow murmur, high output cardiac failure
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
13
Q
PBS of IDA
A
microcytic hypochromic RBC (d/t dec Hgb production or faulty function)
14
Q
response of iron therapy in 12-24hrs
A
subjective improvement (increase appetite, decrease irritability
15
Q
response of iron therapy in 36-48hrs
A
initial bone marrow response (erythroid hyperplasia)
16
Q
response of iron therapy in 48-72hrs
A
reticulocystosis w/c peak at 5-7 days
17
Q
response of iron therapy in 4-30 days
A
inc hgb level
18
Q
response of iron therapy in 1-3 months
A
repletion of iron stores
19
Q
treatment of IDA
A
Elemental iron 4-6mg/kg TID x 8 weeks
20
Q
hallmark of aplastic anemia
A
peripheral pancytopenia with marrow hypoplasia or aplasia
21
Q
moderate aplastic anemia
ANC:
PC:
corrected retic ct:
A
ANC: 500-1000/mm3
PC: 20K-100K/mm3
retic ct: <1%
22
Q
severe aplastic anemia
ANC:
PC:
corrected retic ct:
A
ANC: <500
PC: <20K
retic ct: <1%
23
Q
treatment of choice for aplastic anemia
A
allogenic hematopoietic stem cell transplant
24
Q
TOC for aplastic anemia if w/o HLA-matched sibling or donor
A
immunosuppresive therapy with horse anti-thymocyte globulin and cyclosporine
25
MC inherited aplastic anemia
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
26
diagnostic criteria of Shwachman-Diamond syndrome
exocrine pancreatic insufficiency & variable hematologic cytopenia d/t bone marrow failure
27
Criteria of Diamond Blackfan Syndrome
<1yo
macrocytic anemia w/ no other cytopenia
reticulocytopenia
normal marrow cellularity w/a paucity of erythroid precursors
28
mainstay of therapy for Diamond Blackfan Syndrome
steroids
29
treatment of shwachman diamond syndrom
oral pancreatic enzyme replacement
fat soluble vitamins
BT, G-CSF
hematppoietic stem cell transplant
30
MC inherited enzymatic disorder
Glucose-6-Phosphate Dehydrogenase Deficiency
- x linked
- most important dse of HMP pathway
31
clinical syndrome of G6PD
- episodic hemolytic anemia induced by infection, drugs, fava beans
- spontaneous chronic non-spherocytic hemolytic anemia
32
PBS of G6PD deficiency
low hgb
heinz bodies
anisopoikilocytosis
bite cells
33
Manifestation of Alpha Thalassemia
Hydrops Fetalis w/ Barts Hgb: SEVERE MICROCYTIC anemia
Thalassemia Major: MOD MICROCYTIC anemia
Thalassemia Minor: MILD MICROCYTIC anemia
Silent Carrier: Normal hgb and MCV
34
Manifestation of Beta Thalassemia
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
35
PBS of thalassemia
microcytic hypochromic RBC
target cell
Heinz bodies
36
MC cause of hemolytic anemia d/t red cell membrane defect
Hereditary Spherocytosis
| - d/t abnormalities of ankyrin & spectrin (proteins involved in RBC cytoskeleton)
37
confirms presence of fragile sphere shaped RBCs
osmotic fragility test
38
PBS of hereditary spherocytosis
spherocytes
39
clinical hallmark of sickle cell dse
vasoocclusive phenomena & hemolysis
40
skull radiograph of sickle cell dse
crew cut or hair on end appearance
41
PBS of sickle cell dse
Howell Jolly Bodies & sickle cell
42
preferred diagnosis for sickle cell dse
high performance liquid chromatography
43
MC acquired red cell aplasia
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
best documented viral cause of RBC aplasia assoc w/ chronic hemolysis
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
macrocytic anemia
neutropenia
thrombocytopenia
marrow with ringed sideroblasts
Pearson Marrow - Pancreas Syndrome
- failure to thrive
- marrow failure in neonatal period
- exocrine pancreas deficiency
- muscle and neurologic impairment
- early death
46
absolute retic ct of Anemia of Chronic Dse
normal to low
| PBS: normochromic normocytic
47
treatment for anemia of renal dse
EPO & iron
- mild hemolysis, dec EPO production d/t damaged renal cells
48
duration of physiologic anemia of infancy
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
spontaneous resolution of physiologic anemia in prematurity
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
ineffective erythropoiesis: premature cell death, decrease output of RBC from marrow leading to anemia
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
normal infant daily requirement of folic acid
25-35 ug/day
52
peak incidence of megaloblastic anemia 2 to folate deficiency
4-7 months
53
causes of folate deficiency
malabsorption and chronic diarrhea
phenytoin, phenobarbital, primidone
methotrexate, pyrimithamine, trimethoprim
54
diagnostics for megaloblastic anemia 2 to folate deficiency
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
conditions leading to impaired Vit B12 absorption
```
NEC
regional enteritits
terminal ileum removed
Imerslund-Grasbeck syndrome
absence of transport CHON transcobalamin
```
56
diagnostic for megaloblastic anemia 2 to Cobalamin deficiency
PBS: macrocytic, macroovalocytosis
elevated methylmalonic acid & homocysteine
excessive excretion of methylmalonic acid in urine (nv: 0-3.5mg/24hrs); reliable & sensitive index
57
treatment for neurologic symptoms of vitamin B12 deficiency
vit B12 1mg IM OD x 2wks
58
acquired and hereditary d/o of heme synthesis
sideroblastic anemia
- hypochromic, microcytic
- inc RDW, serum iron and transferrin
- retention of iron in mitochondria: ringed sideroblasts
59
major complication of sideroblastic anemia
iron overload
60
mc molecular defect of of hereditary spherocytosis
spectrin or ankyrin
61
age of functional asplenia
6mos-5yo
62
MC glycolytic enzyme defect as a cause of hemolytic anemia
pyruvate kinase deficiency
63
hallmark of autoimmune hemolytic anemia
(+) direct coombs test
64
Diagnostic criteria for polycythemia rubra vera
```
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
MC and serious congenital coagulation factor deficiency
Hemophilia A or B
- delayed clotting of blood caused by deficiency of clotting factors
- easy bruising, hematoma, hemarthroses when child begin to cruise
66
hallmark of hemophilia
hemarthrosis
67
factor VIII deficiency
hemophilia A
68
factor IX deficiency
hemophilia B
69
factor XI deficiency
hemophilia C
70
most common earliest joint involved
ankle
71
hemophilia A mgt alternative
cryoprecipitate
FFP
emicizumab
72
hemophilia B & C mgt alternative
cryoprecipitate
| FFP
73
MC inherited bleeding Disorder
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
Diagnostics of vWD
- dec vWF
- dec Factor 8
- prolong bleeding time
- abnormal platelet adhesion
- inc PTT
- Ristocetin cofactor assay (measures vWF antigen level & activity)
75
Treatment for vWF
- Desmopressin 0.3ug/kg (inc amt of circulating vWF by releasing from storage
- replacement therapy
- antifibrinolytics
- hormonal treatment w/ estrogen in woman
76
Major symptom of vWD
menorrhagia
77
MC cause of acute onset thrombocytopenia in children
Idiopathic Thrombocytopenic Purpura --> immune thrombocytopenia
- 14 wks after viral infection
- spontaneous resolution in 6 months
78
treatment for ITP
IVIG 0.8-1 g/kg/day for 1-2 days
Prednisone 1-4MKD for 2-3 wks
IV anti D therapy
79
Drugs that induce thrombocytopenia
valproic acid
phenytoin
sulfonamide
heparin
80
Pentad of Thrombotic Thrombocytic Purpura
```
Fever
Anemia (microangiopathic hemolytic anemia)
Thrombocytopenia
Renal dysfunction
Nervous system changes
```
81
Diagnostics of TTP
schistocytes, spherocytes, helmet cells
elevated retic ct
thrombocytopenia
elevated BUN, CREA
82
treatment of TTP
plasmapheresis
Rituximab
steroids
splenctomy
83
triad of Kassabach Merrit Syndrome
cavernous hemangioma
severe thrombocytopenia
intravascular coagulation
giant hemangioma with localized intravascular coagulation -->
decrease platelet and hypofibrinogenemia
84
triad of Wiskott Aldrich Syndrome
Eczema
thrombocytopenic hemorrhage
immunologic defect
normal number of megakaryocytes but abnormal morphology
small sized platelets
lymphoreticular malignancies
85
autosomal recessive inherited platelet adhesion defect d/t GP Ib deficiency
Bernard Soulier Syndrome
- moderate thrombocytopenia, large platelet & decreased
- risocetin induced aggregation not corrected by addition of vWF
86
AR, deficiency of platelet fibrinogen receptor GPIIb-IIIa
Glanzmann Thrombastenia
| - normal plt ct, absent aggregation in vitro, absent clot retraction
87
rare AR disorder w/c there is an absence of fibrinogen
congenital afibrinogenemia
- do not bleed as frequent as patients w/ hemophilia
- prolonged PT, PTT & thrombin time
88
dysfunctional fibrinogens
dysfibrinogenemia
tx: FFP, cryoprecipitate
89
MC malignant neoplasm in <15yo
leukemia
90
peak age of ALL
2-6Y/O
91
most important morphologic feature of ALL
FAB L3 subtype
92
manifestation of ALL
- anorexia, fatigue, irritability, intermittent low grade fever, bone & joint pains (lower extremities)
- history of URTI 1-2months prior
- pallor, bruising, epistaxis
