heamtopoeitic stem cells disorders Flashcards
(28 cards)
Causes of aplastic anemia?
autoimmunity
toxins
infection
meds- antithyroid meds PTU and MMZ, NSAIDS anti convulsants gold
Causes of pure red cell aplasia?
parvo B19 thymoma autoimmune disease solid tumors drugd phenytoin isoniazid pregnancy anti EPO ab
Causes of neutropenia inherited?
cyclic neutropenia
benign ethnic neutropenia
severe congenital neutropenia
neutropenia associated with various congenital syndromes glycogen storage disease type Ib
Causes of neutropenia acquired?
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
Treatment AA
under 50 -allogenic stem cell tp
over 50 immunosuppression with anti thymocyte globulin cyclosporine and prednisone
peripheral blood smear of MDS?
macrocytic anemia
hypogranular neutrophils and platelets
symptoms of severe MDS?
blasts >10%
HG less than 80
platelets less than 50
ANC <0.8
MDS treatment?
only cure of MDS is allogenic stem cell transplant
treat cytopenias
Chronic monomyelocytic leukemia?
persistant monocytosis
with other cytopenias
bone marrow shows dysplastic changes
less than 20%blasts
CML chronic myeloid leukemia?
transocation of chromosome 9 and 22 philadelphia chromosone leads to BCR ABL fusion gene
codes for abnormal tyrosine kinase which promotes dysregulated cell proliferation
SYmptoms of CML?
asymptomatic with elevated neutrophils fatigue weight loss abdominal fullness splenomegaly bleeding
Phases of CML?
chronic phase less than 10% blasts
accelerated phase
blast crisis
treament of CML?
tyrosine kinase inhibitors-imatinib, dasatinib, nilotinib
with acccelerated of blast phase allogenic stem cell transplant
adverse affects of TKI?
fluid retention and QTc prolongation
contrindicated during pregnacy
What are causes of secondary erythrocytosis?
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
SYmtpoms of PV?
prurits after warm shower erythromelalgia TIA DVT/PE splenomegaly plethora
Essential thrombocytosis?
platelets greater than 450
50% JAK2 +
Symptoms of ET?
asymptomatic with elevated platelets headache visual distrubance dyesthesia of palms and soles thrombosis a hemorrhage CVA, TIA, MI, digital ischemia, vision loss
treatment ET?
hydroxyurea for those over 60 and those with platelets of 1000
ASA for those with symptoms
Primary myelofibrosis?
worst prognosis of MPN
marrow fibrosis
50% JAK2 +
Symptoms of PMF?
fatigue weight loss fever chills massive splenomegaly Portal HTN abdomial discomfort and early satiety
Treatment PMF?
HSCT allogenic
ruxolitinib for those that are not candidates to control symptoms
Causes of eosinophilia?
collagen vascular disease EGPA helminthic infection idiopathic neoplasia lymphomas and MPN allergy atopy and asthma
Acute myeloid leukemia?
clonal myeloid proliferation and abnormal delayed differentiation with blasts >20% in bone marrow or blood
