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Granulopoiesis in Infantile Genetic Agranulocytosis In vitro Cloning of Marrow Cells in Agar Culture
Author(s) -
Olofsson Tor,
Olsson Inge,
Kostmann Rolf,
Malmström Stig,
Thilen Astrid
Publication year - 1976
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1976.tb01111.x
Subject(s) - granulopoiesis , bone marrow , in vitro , biology , cloning (programming) , agar , immunology , colony stimulating factor , haematopoiesis , andrology , microbiology and biotechnology , medicine , stem cell , genetics , computer science , bacteria , programming language
A 3‐year‐old boy and a 2‐year‐old girl with infantile genetic agranulocytosis have been studied by in vitro cloning of bone marrow cells in agar culture. The patients display a normal concentration of colony forming cells and the morphological maturation is identical with that of control marrow cultured in vitro. The marrow cells of the patients show some degree of auto‐stimulation indicating that endogenous production of colony stimulating factor is operating. As an inverse relationship is expected between the peripheral neutrophil count and the percentage of marrow colony forming cells in S‐phase a high percentage was expected. On the contrary, we find that the percentage of colony forming cells in S‐phase is extremely low indicating a genetic unresponsiveness of granulopoietic precursor cells to feed back regulation in infantile genetic agranulocytosis.