[Myelodysplastic syndrome in a patient with familial Pelger-Huet anomaly].
This paper reports on a patients with congenital Pelger-Huet anomaly who developed myelodysplastic syndrome (MDS). A 45-year-old female was referred for investigation of pancytopenia of 6 months' duration. Hereditary Pelger-Huet anomaly was diagnosed by family study 7 years prior to admission. On admission, Hb was 6.5 g/dl, Ht 19.9%, Platelets 1.8 x 10(4)/microliters, and WBC 1,200/microliters with 2% myelocytes, 9% metamyelocytes, 14% bands, 2% segmented neutrophils, 58% lymphocytes and 5% monocytes. Most of the granulocytes were Pelger-Huet type with strikingly clumped nuclear chromatin. Bone marrow aspirate demonstrated 3.6% blasts and dysplastic changes including megaloblastoid features in erythroid series and micro-megakaryocytes compatible with refractory anemia, a subtype of MDS. The association of hereditary Pelger-Huet anomaly and MDS is discussed.
Hiraga
H
H
Department of Immunohematology, Kobe City General Hospital, Hyogo.
Yabe
H
H
Nagai
K
K
Nakayama
S
S
D000753:Anemia, Refractory / Q000097:blood / Q000209:etiology; D005260:Female; D006801:Humans; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000097:blood / Q000209:etiology*; D010381:Pelger-Huet Anomaly / Q000097:blood / Q000150:complications*