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Myelokathexis: a rare form of chronic benign granulocytopenia
Author(s) -
Bassan Renato,
Viero Piera,
Minetti Bruno,
Comotti Benedetto,
Barbui Tiziano
Publication year - 1984
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1984.tb06065.x
Subject(s) - medicine , bone marrow , neutropenia , rare disease , myeloid , pathology , hyperplasia , disease , pediatrics , immunology , chemotherapy
SUMMARY A case of childhood chronic neutropenia due to myelokathexis is reported. This extremely rare condition (the fourth case in the literature) has been diagnosed from the characteristic bone marrow abnormalities in the neutrophils. Myelokathexis (kathexis‐retention) is a form of childhood granulocytopenia characterized by marked degenerative changes in mature neutrophils and considerable hyperplasia of the bone marrow myeloid cell series. The disease is rare and, since the first description by Zuelzer in 1964, only two additional cases have been reported (Krill et ah 1964; O'Regan et al 1977). We wish to describe the first case observed in Italy which is also the first reported outside U.S.A.