Bone marrow disorders Flashcards
2 disorders of bone marrow failure
aplastic anemia
MDS
3 myeloproliferative disorders
PV
ET
primary myelofibrosis
features of aplastic anemia
pan cytopenia
- history - fatigue SOB
- bone marrow empty
Treatment of Aplastic anemia
- immunosuppressive therapy
- allogeneic stem cell transplant
who gets aplastic anemia
disease of children and young adults
Median age of diagnosis of MDS
~70 yrs
Diagnosing aplastic anemia
- bone marrow aspirate and biopsy
How does MDS present?
anemia
cytopenia - progressive
Diagnosing MDS
- blood film - ring sideroblasts
- blast counts
cytogenetics del 5q
Ring sideroblasts classic feature of
MDS
Treating low risk MDS
Fix cytopenia to ameliorate symptoms
Treating HIGH risk MDS
delay or prevent AML, extend life span
MDS can progress to
AML
Bone marrow in Aplastic anemia vs MDS
empty vs FULL
peak age for myeloproliferative disorders
55-70 yo
97% of patients with PV hae
JAK2 mutations V617F
Diagnosis of PV
dehydration
high Hb, High red cell
Splenomegaly
JAK2 mutation
Treatment of PV
phlebotomy
oral chemo hydroxyurea
jak 2 inhibitor
Diagnosis of ET
- rule out thrombocytosis due to other cause
- look for JAK2 mutation, cmpl
if you have ET and are thrombotic
give ASA
and hydroxyurea - same for if bleeding
- patient with tear drop red cells
- reticulin positive stain
- hepatosplenomegaly
- blasts in peripheral blood
Primary myelofibrosis
Life expectancy with
PV
ET
MF
Decades
same as healthy person
MF - steady deterioration, 2-15 years
Diagnosis of MF
- DRY tap
JAK2 mutation in 70%
CALR mutation in 25%
Treatment of MF
- transfusion
- splenectomy - risky
SCT - risky
JAK2 inhibitors
Big spleen with high Hb
PV?
pancytopenia
BM failure?
acute leukemia
get a bone marrow
low MCV low Hb
iron deficiency not ET
hypogranular neutrophils - dysplastic
- macrocytic - Increased MCV
-
- MDS!
Palpable spleen tip
- elevated white count
elevated platelets very high
think ET
