Hematology/ ONcology Flashcards
(135 cards)
1
Q
3 possibilities for causes of anemia
A
reduced production
increased destruction
loss of RBCs
2
Q
in what type of anemia will MCV be low?
A
iron deficiency
3
Q
what type anemia is due to red cell aplasia?
A
Diamond Blackfan anemia
4
Q
is “normal” reticulocyte count in the face of anemia normal?
A
No, it is abnormal
5
Q
how to you tx diamond blackfan anemia?
A
steroids
BMT is curative
6
Q
if reticulocyte count is increased and there is anemia what is happening?
A
hemolysis
blood loss
7
Q
what are intrinsic problems that cause hemolysis leading to anemia?
A
membrane defects- hereditary spherocytosis
enzyme-G 6PD deficiency
Hemoglobinopathies
8
Q
what is the most common childhood hematologic problem?
A
anemia
9
Q
what does jaundice with anemia indicate?
A
hemolysis
10
Q
where do all RBCs go to die?
A
spleen
11
Q
presents with Pallor, fatigue, dizziness, poor growth, exercise intolerance
Tachycardia, murmur, headache, syncope
A
anemia
12
Q
anemia w/ lymphadenopthy can indicate what?
A
leukemia
HIV
JRA
13
Q
anemia with splenomegaly can indicate what?
A
leukemia
sickle cell
hemoglobinopathy
spherocytosis
14
Q
what are the three cell lines?
A
red cells
white cells
platelets
15
Q
when 2 cell lines are depressed what is there a higher likelihood for?
A
leukemia
16
Q
what do schistocytes indicate?
A
HUS
17
Q
what can blister cells indicate?
A
G6PD
18
Q
should iron deficiency show up early in life?
A
no, have iron stores from mom for first 5-6 months of life
19
Q
what causes iron deficiency anemia in kids 6-24 months
A
poor dietary intake (ie cows milk)
20
Q
tx for iron deficiency anemia
A
6 mg/ kg/ day divided TID
21
Q
why does cows milk lead to iron deficiency anemia?
A
calcium in cows milk binds the iron
22
Q
what are 2 macrocytic anemias?
A
Folate and B 12 deficiency
23
Q
what does irritable, weakness, paresthesias, unsteady gait indicated deficiency in
A
Vit B 12
24
Q
congenital aplastic anemia. Will have congenital anaomlaies, thrombocytopenia. diagnosed in 2-10 year olds
A
fanconi’s
25
just affects red cell lines. shows up at birth- 1 year.
diamond black-fan
26
what are 2 congenital anemias
fanconi
| diamond black fan
27
what can cause acquired anemias
idiosyncratic rxn to meds
after an infection
idiopathic (mostly)
28
in acquired anemias are there HSN or jaundice?
No
29
what causes isoimmunization hemolysis?
Rh/ABO/ minor blood groups
30
what are congenital hemolysis?
sphereocytosis
G6PD deficiency
pyruvate kinase deficiency
hemoglobinopathies
31
autosomal dominant. Managed with splenectomy. cells look like spheres.
Hereditary spherocytosis
32
why do you take the spleen out with hereditary spherocytosis
so the spleen stops killing the red blood cells
33
Most frequent inherited enzyme defect
X-linked recessive (males)
Anemia, hyperbilirubinemia* jaundice
Episodic hemolysis with oxidative stress
G6PD deficiency
34
what is the 2nd most commonly inherited enzyme defect
pyruvate kinase deficiency
35
when do symptoms of beta thalassemias show up?
4 months due to fetal hemoglobin
36
when do symptoms of sickle cell onset?
3-4 months
37
what will you have with sickle cell dz?
splenomegaly due to sequestration
| recurrent episodes of vasoocclusion and tissue ischemia
38
what should be given for prophylaxis for sickle cell dz?
penicillin (pneumococcal, meningococcal, H. flu)
39
what are 3 general categories of platelet disorders
Decreased production
Increased consumption
Impaired function
40
what are 3 disorders of coagulation
Hemophilias
Vitamin K deficiency
DIC
41
what measure the intrinsic pathway?
aPTT
42
what measures the extrinsic pathway?
PT
43
kids show up with a lot of petechiae and purpura what do you suspect?
ITP
44
what causes decreased production leading to thrombocytopenia
```
infiltrative diseases (affect bone marrow)
congenital production defects
viral infection?
```
45
large vascular venous malformation
Kasabach-Merritt syndrome
46
what cause increased consumption leading to thrombocytopenia (more common)
ITP
DIC
Kassabach-Merrit syndrome
47
what causes thrombocytopenia by impaired function
```
Von Wilebrand dz
class test- bleeding time
```
48
in peds what is the most common bleeding disorder?
ITP
49
what are 3 acquired bleeding disorders?
DIC
liver dz
henoch Schonlein Pupura (HSP)
50
what age range is ITP most common in?
2-5
51
what does ITP most often follow?
viral infection
52
how do you tx ITP
steroids
| IVIG in severe situations
53
will there be LAD or HSM with ITP
No
54
what will platelet counts be below with ITP
<10,000
55
What will be increased in the bone marrow with ITP?
megakaryoctyes
56
factor VIII deficieicny is what?
Hemophilia A
57
factor IX deficieincy is what
hemophilia B or
| Christmas Dz
58
hemophilias will have prolonged what?
aPTT
59
Tx for hemophilia A (classic)?
Factor VIII
| desmopressin releases endothelial stores of factor VIII and vWf
60
Tx for hemophilia B (christmas dz)?
Factor IX
61
Most common inherited bleeding disorder among Caucasians – autosomal dominant
Von Willebrand Dz
62
Tx for Von Wilebrand Dz?
Desmopressin (causes release of VWF from endothelial stores)
63
what is an autoimmune mediated vasculitis.
Henoch schonlein purpura
64
what causes cholestasis?
IV fluids (TPN live on trashes your liver)
65
what contains all the clotting factors and can help with DIC?
fresh frozen plasma
66
what do you give for a low fibringoen?
cryoprecipitate
67
what is a physiologic cause of unconjugated hyperbilirubinemia?
breast milk jaundice
68
what enzyme catalyzes bilirubin conjugation in liver.
Glucuronyl transferase (low levels at birth)
69
what is a condition where an infant often has polycythemia at birth?
trisomy 21
70
caused by Abnormal gene promoter leading to decreased transcription of mRNA less enzyme
Gilbert's syndrome
71
what are sxs of gilbert's syndrome?
life-long episodes of mild juandice
| enhanced neonatal juandice
72
tx for gilbert's syndrome
no tx except in neonate w/ excessive hyperbilirubinemia
73
who is Gilbert's syndrome more common in?
males
74
are LFTs normal with Gilberts
yes, except direct bili
75
syndrome with no enzyme activity leading to severe unconjugated hyperbilirubinemia starting at th-5th day of life
Type 1
| Crigler-Najjar Syndrome
76
How do you tx type 1 crigler-najiar syndrome?
liver transplant
77
what type of Crigler-Najjar Syndrome has diminished activity?
Type 2
78
how do you tx Crigler-Najjar Syndrome
| type 2?
phenobarbital
79
conjugated hyperbilirubinemia is a sign of what?
liver dz
80
what happens with biliary atresia
conjugate the bilirubin but you can't excrete it. Will have impeded bile acid flow
81
what does a wide mediastinum indicate?
thymus, lymphadenopathy
82
2 main cancers in peds population
leukemias
| brain tumors
83
what is the most common death by disease in children?
cancer
84
what are the 2 main things to do for a CA workup?
CBC with diff
| peripheral blood smear
85
what is the most common of the leukemias?
acute lymphoblastic leukemia (ALL)
86
when is ALL common?
ages 2-10
87
what conditions have a higher incidence of ALL
T21
NF type 1
Fanconi's anemia
88
what will you see with ALL on chest xray?
mediastinal widening or anterior mediastinal mass or tracheal compression from lymphadenopathy.
89
are bone marrow transplants (HSCT) common with ALL?
no, chemo usually cures
90
which has a worse prognosis- ALL or AML?
AML
91
what leukemia can have translocation of chromosome 9 and 22 (Philadelphia chromosome)
Chronic Myelogenous Leukemia
92
what is the only curative intervention for CML?
HSCT
93
where do lymphomas arise?
lymph nodes
thymus
spleen
94
what is the most common lymphoma?
Hodgkin disease
95
where will you find a mass with hodgkin lymphoma?
cervical, supraclavicular adenopathy or mediastinal
96
what cells will be present with hodgkin?
Reed Sternberg cells (malignant B cells)
97
Tx for hodgkin dz?
chemo + los dose radiation
98
what will a child with hodgkin present with?
```
fatigue
cough
SOB
anorexia
weight loss
fever
night sweats
```
99
are hogkin's or NH more malignant?
NK
100
Tx for NH lymphoma?
chemotherapy
101
what is the second most common cancer in kids?(more common solid tumor)
brain tumors
102
what is the classic triad of brain tumors in kids?
morning headache, vomiting, papilledema
103
what is the most common brain tumor of childhood?
astrocytoma (also known as gliomas)
104
symptoms that go along with mass in supratentorial area?
seizures
hemiplegia
focal neurological signs
105
What symptoms will you have with an craniopharyngioma (midline tumor)
bitemporal hemianopsia
pituitary failure (growth failure)
diabetes insipids
weight gain
106
what would a tumor in the cerebellar and IVth ventricle present with?
truncal ataxia
coordination difficulties
abnormal eye movements
107
What would a brainstem tumor present like?
cranial nerve defects
pyramidal tract signs
ataxia
often no raised ICP
108
does cerebellar astrocytoma have high or low cure rate?
high cure rate
109
what brain tumors have a very poor prognosis?
brainstem gliomas
| glioblastoma
110
with kidney problem what diagnostic should you start with?
Ultrasound
111
what does a neuroblastoma do that a nephroblastoma doesn't?
crosses the midline
112
what is the most common solid neoplasm outside the CNS?
Neuroblastoma
113
when you hear abdominal pain and masses what should you suspect?
neuroblastoma
114
Tx for neuroblastoma
surgical resection with chemo
115
what is the definitive diganostic tool for neuroblastoma?
biopsy
116
large tumor in the kidney that doesn't cross the midline.
Wilms tumor
117
what is the 2nd most common abdominal tumors in children?
Wilms tumor
118
when are Wilm tumors common (age wise)?
2-5, unusual after age 6
119
who can wilms tumors been more common in?
Kids with hypospadias, GU malformations and others
120
is a Wilms tumor painless or painful?
painless
121
Tx for wilms tumor.
surgical resection/ exploration then chemo
122
what other physical symptoms may be present with a Wilms tumor?
hematuria and HTN
123
neuroectodermal malignancy” arising from embryonic retinal cells
Retinoblastoma
124
most common sign of retinoblastoma.
white pupillary reflex
125
tx for retinoblastoma
radiation, enucleation (take out eye)
| prognosis depends on spread beyond globe
126
when is a retinoblastoma usually diagnosed?
before age 3
127
Diagnostics for retinoblastoma
MRI
128
most common soft tissue sarcoma in childhood
Rhabdomyosarcoma
129
when are the 2 peaks for sarcomas in children?
2-5 years and adolescents
130
most common locations for sarcomas in younger patients.
head, neck, and GU
131
most common locations for sarcomas in older patients
extremities
trunk
male GU
132
Diagnostics for Rhabdomyosarcoma
Xrays
Ct
MRI
(look for tumor and mets)
133
Tx for rhabdomyosarcoma
surgery
chemo
radiation
134
when are osteosarcomas and ewing sarcomas most often seen
growth spurts
135
is osteosarcoma or Ewing sarcoma more commonly seen?
osteosarcoma
