DR Medhat MRCP

Myelofibrosis
- Myeloproliferative disorder due to ↑ proliferation of fibroblasts.
• Pathogenesis :
Hyperplasia of megakaryocytes → ↑ platelet derived growth factor →
stimulate fibroblasts
Hematopoiesis develops in the liver and spleen
• Features :
Elderly person with symptoms of anemia e.g. Fatigue (the most common presenting
symptom).
Massive splenomegaly.
Hypermetabolic symptoms: weight loss, night sweats etc
• Laboratory findings
Anemia
High WBC and platelet count early in the disease
'Tear-drop' poikilocytes on blood film
Unobtainable bone marrow biopsy : 'dry tap' therefore trephine biopsy needed
High urate and LDH (reflect ↑ cell turnover).
Myelodysplastic syndrome
- Acquired disease where the BM myeloid stem cells fail to produce mature healthy
blood cells→↑myeloblasts > 2-3 % but < 20% (i.e between normal & leukemic range)
- Myeloid = BM myeloid cells , dysplasia = abnormal cells & it`s characterised by :
Peripheral blood Pancytopenia (cells die early by apoptosis)
Hypercellularity of BM (unlike in aplastic anemia).
Morphological abnormalities in ≥ 1 lineage of haemopoietic cell lines growth
- It → AML if a gene mutation occur leading to uncontrollable blast cells proliferation.
• C/P : most commonly in elderly with multiple comorbidity.
Refractory anemia (main presenting symptoms (80%)
Recurrent infections (leucopenia)
due to pancytopenia.
Bleeding tendency (thrombocytopenia)
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