Myeloproliferative Disorder Flashcards
Myeloproliferative syndrome defination ??
- proliferative neoplastic disorder
- uncontrolled proliferation of abnormal progenitor cells
- which are capable of producing myeloid,erythroid,megakaryocytic series
names of myeloproliferative disease ?
- polycythemia rubra vera
- CML
- myelofibrosis
- essential thrombocythemia
what is myelofibrosis ??
fibrosis & collagen formation in the marrow
CBC of myelofibrosis ?
- Hb% -
- PCV -
- RBC -
- WBC-
- P -
- ESR +
PBF of Myelofibrosis ?
- RBC = N-N-A-P-tear drop cells - rouleux formation
- WBC -
- P -
BM of myelofibrosis ?
- C Acellular
- M/E Normal
- E -
- G-
- M-
other test for myelofibrosis ?
- folic acid -
- uric acid +
- leukocyte alkaline phosphatse score +
Polycythemia-Def ?
elevated levels of all blood components
when it is True Polycythemia ?
Elevation in all blood count
types of True polycythemia ?
- P rubra vera
- secondary P
when it is relative Polycythemia ?
reduction in the plasma volume
causes of pseudo-P ?
- stress
- cigarette
- burns
- dehydration
- reduced fluid intkae
CBC of Polycythemia Rubra Vera ?
- Hb% +
- PCV +
- RBC +
- WBC +
- P +
- ESR -
BM of PRV ?
- C= +
- M/E = Normal
- E - ERYTHROID hyperplasia
- G - HYPERACTIVE
- M -Hyperactive
Others test for PRV ??
- janus associated kinase- 2
- loss/gain of C-9
- NAP +
- serum erythropoietin -
Causes of PRV ?
- Hypoxia
- high altitude
- heart disease
- heeavy cigarette smoker
- high affinity to Hb
- polycystic kidney disease
- renal d
- carcinoma
- beningn familial polycythemia
- 2,3 biphosphoglycerate deficiency
Diagnosis Triad of PRV ??
- Splenomegaly
- elevated Hb with leukocytosis & thrombopcytosis
- dusky brick red colour of the face = Ruddy cyanosis
WHO criteria major for PRV ?
- JAK-2 mutation
- Hb% male >18gm/dl
- female >16gm/dl
minor criteria for PRV ?
- erythropoitin -
- inc bone marrow proliferation
ss of PRV ?
- thrombosis
- DVT
- HTN
- PVD
- Hemorrhage
- hepatosplenomegaly
- headache
- dizziness
- pruritis
- poor vision
myelofibrosis VS CML ?
CML = philadelphia chromosome
MF = LAP score +
WBC count + in CML
Blast cell in CML
which type red cell inc in p ?
erythropoieitn