Chronic blood disorders

Question 1

what are chronic myeloproliferative disorders

Answer 1

myeloid progenitor stem cell disorders of the bone marrow

Question 2

what are three types of chronic myeloproliferative disorders and their prognosis

Answer 2

polycythemia vera - good
essential thrombocytosis - good
idiopathic myelofibrosis - bad

Question 3

what is polycythemia vera, features, diagnosis and treatment

Answer 3

increased RBC’s, loss of fat spaces in bone and may increased neutrophils

itching, gangrene, splenomegaly, gout

test for JAK2 mutation
low EPO = PV

aspirin, venesection - good prognosis

Question 4

what is essential thromcytosis, diagnosis and treatment

Answer 4

increased platelet count - clusters in bone marrow

test for JAK2 or CALR mutation, platelets higher than 450

aspirin and cytoreduction (hydroxycarbamide)

Question 5

what is idiopathic myelofibrosis, features, diagnosis and treatment

Answer 5

bone narrowing scarring = low blood count and splenomegaly

pancytopenia - low RBC, WBC and platelet
massive splenomegaly

tear shaped RBC, JAK2 or CALR mutation

treat with BM transplant, JAK2 inhibitor

Question 6

what are the causes of splenomegaly

Answer 6

CHICAGO
C - cancer 
H - haematological 
I - infection 
C - congestion 
A - autoimmune 
G - glycogen storage disorder 
O - other

Question 7

what is chronic myeloid leukaemia, features, cause and treatment

Answer 7

neoplasm of myeloid progenitor causing leucocytosis in the blood - presence of myelocytes in blood (precursor to neutrophils)
splenomegaly

chromosome t(9;22) translocation 
philadelphia chromosome 

treat chronic - imatinib (BCR-ABL TK inhibitor)
acute - chemo or BM transplant

acute phase = poor prognosis