HI FLOWERS: Today I have a immunphenotyping of a patient,a male,4 years, the sample was bone marrow (als periphereal blood), and the diagnosis was Kostmann`s neutropenia. Also the patient is under treatment with G-CSF. In the immunophenotyping I found a few things that not are clear for me: the netrophil population have a normal FSC but have a low SSC, and also have normal markers for myeloid (CD33, CD13, MPO, TDT), but also have a expression of CD61 (M7?). Also I found a little population of cells with low SSC and Low expression of CD45 (the classical region of blast). This cells was CD19, CD20, HLA-DR, CD10(CALLA), MPO dim, TDT(-), CD61(-), CD34 (-), CD33 (-), CD13 (-), CD14(-). I search information about Kostmann`s syndrome, and the next is the principal: Severe congenital neutropenia (SCN) or Kostmann's syndrome is characterized by a stop in differentiation of myeloid progenitor cells at the myelocytic or promyelocytic stage. with absence of neutrophils in bone marrow (BM) and blood. The pathophysiology of SCN is still unclear. (Exp Hematol 1999 Jun; 27(6):1038-45. Hypotheses of the pathophysiology of SCN include (1) defective production of granulocyte colony-stimulating factor (G-CSF), and/or (2) defective response to G-CSF. (Blood 1991 May; 77(9):1919-22). The administration of granulocyte- colony-stimulating factor was shown to be safe and effective also in reducing infectious episodes in these patients. (Acta Paediatr 1992 Feb; 81(2):133-6). Thus, it is hypothesized that the underlying defect responsible for SCN is based on an abnormal G-CSF-induced signal transduction pathway .eutrophils from SCN patients show an increased autophosphorylation of JAK2 (a nonreceptor tyrosine kinase involved in the signaling pathway of G-CSF )in comparison with that of neutrophils from healthy volunteers. (Blood 1995 Dec; 86(12):4500-5). So, the patient : May be a leukemia ? , M7 ? , lymphoid ? or Does the normal production of bone marrow upon treatment of G-CSF ? Any idea will helpme Thanks JUAN LUIS CASTILLO N. CITOMETRIA DE FLUJO HOSPITAL DEL TRABAJADOR CONCEPCION CHILE PHONE: 56-41-201722 FAX: 56-41-311008 EMAIL: axelyoyi@entelchile.net
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