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Saturday 12 December 2015

An old lady with fatigue and abnormal blood film

A 65-year-old woman consulted her physician for her increasing fatigue from which she suffered for the preceding 3 months. She also noticed a 2-Kg decrease in her body weight. 
Her past history is remarkable for systemic arterial hypertension which was well controlled by lisinopril 10 mg O.D, and hysterectomy 9 years earlier for dysfunctional uterine bleeding.

On examination, she was pale. 
Blood pressure: 125/85 mmHg, pulse rate: 83 (regular), temperature: 37.2 degrees celsius.
Otherwise, the physical examination was unremarkable, including neurological examination.

Lab tests results were significant of low hemoglobin (8 g/dl), low WBC count (2.5x10³ / µL).
Serum ferritin, vitamin B12, and RBC folate were normal.

Blood smear examination showed reduced neutrophil count, with bilobed nuclei, and the presence of promyelocytes.
Some RBCs looked oval in shape, and the platelets were normal.

The bone marrow was hypercellular with features of erythroid hyperplasia and small megakaryocytes.
Ringed sideroblasts were demonstrated using Prussian blue stain.

What's the most likely diagnosis? And how should this patient be treated?


The answer is shown below the next photo







































The most likely diagnosis is myelodysplastic syndrome based on the following findings:

-The age of onset is suggestive
-The absence of iron or vitamin deficiency
-The presence of neutrophils with bilobed nuclei (Pelger-Huet abnormality, shown in the above photo)
-Hypercellular bone marrow with the presence of ringed sideroblasts, and small (dwarf) megakaryocytes



The above mentioned features clearly distinguish myelodysplastic syndrome from:

-Aplastic anemia : hypocellular bone marrow.
-Megaloblastic anemia : folate or vitamin B12 deficiency.
-Myelofibrosis : Massive splenomegaly, leukoerythroblastic blood film with megakaryocytosis, and bone marrow fibrosis.
-Drug induced cytopenias, infection.

Treatment plan:
-Since the patient is hemodynamically stable, blood transfusion is not required at this time.
-Epoetin and G-CSF to stimulate erythropoiesis.
-Filgratism (myeloid growth factor)
-Oral thrombopoietin analogues may not be needed because of her normal platelet count.


NB: For patients with loss of part of the long arm of chromosome 5 (5q- syndrome), treatment with lenalidomide can result in long-lasting cytogenetic remission, and more favorable prognosis.

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