title: Assessing donor chimerism using flow cytometry in paroxysmal nocturnal haemoglobinuria after stem cell transplantation--a case report.
abstract: Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired haemopoietic stem cell disorder arising from somatic mutation of the X-linked PIG-A gene which leads to deficiency of the glycosylphosphatidylinositol (GP1) membrane anchor proteins such as CD 59 (MIRL: membrane inhibitor of reactive lysis) and CD 55 (DAF: decay accelerating factor). Allogeneic peripheral blood stem cell transplant (PBSCT) is a curative mode of treatment in symptomatic PNH patients. Assessment of donor chimerism for PBSCT can be performed by various methods including short tandem repeat loci (STR) and variable number of tandem repeats (VNTR). Flow cytometry, which is much cheaper and faster, also can be used to assess engraftment in patients with PNH. Engrafted patients will show the presence of CD 55 and CD 59 on their red cells and white cells. We describe here the usefulness of flow cytometry in the assessment of donor chimerism following allogeneic PBSCT, in a case of PNH.
authors: Department of Pathology, Faculty of Medicine, Universiti Kebangsaan Malaysia, Jalan Yaacob Latif, Bandar Tun Razak, 56000 Kuala Lumpur, Malaysia. zahratul@mail.hukm.ukm.my
authors: R Z
authors: RZ
authors: Azma
authors: N H
authors: NH
authors: Hamidah
authors: C F
authors: CF
authors: Leong
authors: O
authors: O
authors: Ainoon
authors: S K
authors: SK
authors: Cheong
mesh_terms: D000328:Adult; D001782:Blood Donors; D018958:CD55 Antigens / Q000032:analysis; D018957:CD59 Antigens / Q000032:analysis; D046528:Chimerism; D004912:Erythrocytes / Q000737:chemistry; D005434:Flow Cytometry / Q000379:methods*; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood / Q000235:genetics / Q000628:therapy*; D006801:Humans; D016130:Immunophenotyping; D007962:Leukocytes / Q000166:cytology; D008297:Male; D018895:Microsatellite Repeats; D036102:Peripheral Blood Stem Cell Transplantation; D018183:Transplantation Chimera / Q000235:genetics*; D014184:Transplantation, Homologous
chemical_list: D018958:CD55 Antigens; D018957:CD59 Antigens