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
/ ยตL).
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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