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Hematology JD > heamtopoeitic stem cells disorders > Flashcards

heamtopoeitic stem cells disorders Flashcards

1
Q

Causes of aplastic anemia?

A

autoimmunity
toxins
infection
meds- antithyroid meds PTU and MMZ, NSAIDS anti convulsants gold

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2
Q

Causes of pure red cell aplasia?

A 
parvo B19
thymoma
autoimmune disease
solid tumors
drugd phenytoin isoniazid
pregnancy
anti EPO ab
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3
Q

Causes of neutropenia inherited?

A

cyclic neutropenia
benign ethnic neutropenia
severe congenital neutropenia
neutropenia associated with various congenital syndromes glycogen storage disease type Ib

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4
Q

Causes of neutropenia acquired?

A

autoimmune
SLE RA
associated with immunodeficiency
infection- viral , bacterial TB, fungal histoplasmosis, parasitic malaria ,
meds- NSAIDS , AB, chemo, anticonvulsants, quinine, psychotropic drugs
nutritional - folate B12 def
sequestration and neutrophil magination- dialysis
hematologic disorders- acute leukemia, aplastic anemia, MDS

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5
Q

Treatment AA

A

under 50 -allogenic stem cell tp

over 50 immunosuppression with anti thymocyte globulin cyclosporine and prednisone

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6
Q

peripheral blood smear of MDS?

A

macrocytic anemia

hypogranular neutrophils and platelets

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7
Q

symptoms of severe MDS?

A

blasts >10%
HG less than 80
platelets less than 50
ANC <0.8

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8
Q

MDS treatment?

A

only cure of MDS is allogenic stem cell transplant

treat cytopenias

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9
Q

Chronic monomyelocytic leukemia?

A

persistant monocytosis
with other cytopenias
bone marrow shows dysplastic changes
less than 20%blasts

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10
Q

CML chronic myeloid leukemia?

A

transocation of chromosome 9 and 22 philadelphia chromosone leads to BCR ABL fusion gene
codes for abnormal tyrosine kinase which promotes dysregulated cell proliferation

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11
Q

SYmptoms of CML?

A 
asymptomatic with elevated neutrophils
fatigue
weight loss
abdominal fullness splenomegaly
bleeding
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12
Q

Phases of CML?

A

chronic phase less than 10% blasts
accelerated phase
blast crisis

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13
Q

treament of CML?

A

tyrosine kinase inhibitors-imatinib, dasatinib, nilotinib

with acccelerated of blast phase allogenic stem cell transplant

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14
Q

adverse affects of TKI?

A

fluid retention and QTc prolongation

contrindicated during pregnacy

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15
Q

What are causes of secondary erythrocytosis?

A

polycythemia vera- primary
mediated by hypoxemia- COPD, sleep apnea, congential HD, intrapulmonary shunting, elevated altitude
mediated by ectopic or excessive EPO- RCC, RAS, HCC, uterine fibroids
high oxygen affinity hemoglobin

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16
Q

SYmtpoms of PV?

A 
prurits after warm shower
erythromelalgia
TIA
DVT/PE
splenomegaly
plethora
17
Q

Essential thrombocytosis?

A

platelets greater than 450

50% JAK2 +

18
Q

Symptoms of ET?

A 
asymptomatic with elevated platelets
headache
visual distrubance
dyesthesia of palms and soles 
thrombosis a
hemorrhage
CVA, TIA, MI, digital ischemia, vision loss
19
Q

treatment ET?

A

hydroxyurea for those over 60 and those with platelets of 1000
ASA for those with symptoms

20
Q

Primary myelofibrosis?

A

worst prognosis of MPN
marrow fibrosis
50% JAK2 +

21
Q

Symptoms of PMF?

A 
fatigue
weight loss
fever 
chills
massive splenomegaly
Portal HTN
abdomial discomfort and early satiety
22
Q

Treatment PMF?

A

HSCT allogenic

ruxolitinib for those that are not candidates to control symptoms

23
Q

Causes of eosinophilia?

A 
collagen vascular disease EGPA
helminthic infection
idiopathic
neoplasia lymphomas and MPN
allergy atopy and asthma
24
Q

Acute myeloid leukemia?

A

clonal myeloid proliferation and abnormal delayed differentiation with blasts >20% in bone marrow or blood

25
Q

symptoms of AML?

A 
anemia, thrombocytopenia, functional neutropenia
petechiae
epistaxis
mucosal hemorrhages
Auer rods
26
Q

acute promyelocytic leukemia?

A

poorly differentiated leukocytes with distinctive primary granules that lead to coagulopathy
chromosomal transformation t15, 17
treated with all trans retanoic acid to cure
DIC

27
Q

Treatment of AML?

A

induction with anthracycline( daunorubicin) and cytarabine

to ablate bone marrow

28
Q

Acute lymphoblastic leukemia?

A

more common in children

philadelphia chromosome in 20-30%

Hematology JD (3 decks)

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