myeloid disorders Flashcards
what are the two types of myeloproliferative neoplasms ?
BCR-ABL +ve
BCR-ABL -ve
BCR-ABL +ve ?
chronic myeloid leukemia
at what stage are most patients diagnosed ?
at their chronic stage
what are the three phases of chronic myeloid leukemia ?
chronic phase
accelerated phase
blast phase ( worst prognosis)
what is the prognosis of each of the three stages of CML?
chronic phase - responsive to treatment
accelerated phased - decreased responsiveness
blast phase - resistant to treatment
what are the symptoms in the chronic phase of CML ?
may be symptomatic OR fatigue weight loss abdominal pain or discomfort night sweats
accelerated phase symptoms ?
myelofibrosis
progressive splenomegaly
what is the treatment for CML ?
tyrosine kinase inhibitors (TKI) (Ruxolitinib)imatinib
what is a deep molecular response ?
reduction in BCR-ABL expression (MR 4-MR5)
what are the types of BCR-ABL -ve malignancies ?
there are the classic and non classic forms
what are the classic types of BCR-ABL -ve neoplasms ?
essential thrombocythemia ( over production of platelets) polycythemia vera (over production of RBCs) primary myelofibrosis
what are the non classic types of BCR-ABL -ve neoplasms?
mastocytosis ( accumulation of mast cells)
chronic neutrophilic leukemia
chronic eosinophilic leukemia
what are the clinical complications of BCR-ABL -ve MPNs?
major thrombosis
splenomegaly
microvascular symptoms
how is essential thrombocythemia diagnosed ?
a platelete count of more than 450 for at least 2 months with no other cause
what are the risk factors of thrombocythemia ?
previous thrombosis
how would you treat essential thrombocythemia ?
acording to the risk
High risk: aspirin and hydroxyurea
intermediate risk: just aspirin
low risk : just aspirin
how would you diagnose polycythemia vera?
hb >16.2 with no other cause
what is the main cause of polycythemia vera ?
JAK-2 mutation
what can you give a polycythemia vera patient ?
Hydroxyurea and aspirin may help
and venesection
what is the problem with using hydroxyurea in Polycythemia vera ?
possible risk of transforming to AML
what is myelofibrosis ?
replacement of bone marrow by fibrosis
what are the clinical features of MF ?
anemia
splenomegaly
abnormal RBC
what is the treatment for MF?
stem cell transplantation
blood transfusion
radiation therapy
splenectomy
what are some preexisting disorders that may be accompanied with acute myeloid leukemia ?
myelodysplastic syndrome
fanconi’s anemia
down syndrome
what kind of anemia is in fanconi’s anemia
aplastic anemia
what are the clinical manifestations of acute myeloid leukemia ?
related to problems caused by bone marrow failure
leukemic cells infiltrating patients organs ( splenomegaly hepatomegaly, bone pain)
hyperurecemia
tumor lysis syndrome
coagulopathy
what is tumor lysis syndrome ?
a group of metabolic disturbances including : Hyperkalemia Hyperphosphataemia Hyperuricaemia Hypocalcaemia
how is AML differentiated from ALL ?
positive sudan black and myeloperoxidase stain in AML
what is present in bone marrow aspirate in AML ?
hypercellular
more than 20% blast cells
presence of Auer rods
what are the methods used too diagnose AML
immunophenotyping
cytogenetics/FISH
Molecular genetics
what are the prognostic parameters in AML
Age
Performance status poor
cytogenetics
response to induction chemotherapy
what kind of supportive care should be offered for AML patients
transfusion
infection control
lymphopenia
what is the purpose of combination therapy ?
reduce drug resistance
reduce drug toxicity
interrupt cell growth at multiple points in the cell cycle
what to do after remission is achieved ?
intensification of therapy
high dose therapy
same drugs at higher doses
what are the examples of BCR-ABL negative diseases ?
primary myelodysplasia
polycythemia vera
what is the gene abnormality associated with CML ?
translocation of 9 22
t(9;22)
ph chromosome
how is acute promyelocytic leukemia confirmed through cytogenetics ?
RARA gene
t(15;17)
