hematology
Contributions
Type: Publication Only
Background
Flow cytometric immunophenotyping has become the gold standard for the diagnosis and monitoring of Paroxysmal hemoglobinuria(PNH). PNH results from acquired somatic mutation of the phosphatidylinositol glycan complementation class A (PIG-A) gene, leading to partial or complete absence of the glycosylphosphatidylinositol (GPI) anchor that is responsible for linking a large number of proteins to the cell membrane.
Aims
The aim of our work is to detect and quantify by flow cytometry (FC) PNH clones in aplastic anemia (AA) patients.
Methods
From January 2001 to december 2013,we have evaluated 103 patients with aplastic anemia. The presence of a PNH clone was assayed by peripheral blood flow cytometry on granulocytes(CD16) and monocytes(CD14). The diagnosis of PNH was retained by the presence of a deficit clone greater than or equal to 50% of white blood cells. The PNH clone is considered small if > 5%, and major if >30%.
Results
Among all AA patients, 15% patients had the PNH clone. Ham's test was performed in ¾ of cases. Anemia was present in all patients with PNH-AA syndrome. Among them,80% had severe aplastic anemia. Bone marrow transplantation (BMT) concened 21 % of patients with PNH-AA syndrome. three patients with PNH-AA syndrome died by severe thrombotic accident, progression to acute leukemia and severe anemia.
| Patients number | Percentage |
Major HPN clone | 5 | 5% |
Small HPN clone | 10 | 10% |
No clone HPN | 88 | 85% |
Total | 103 | 100% |
Summary
The flow cytometry on granulocytes is a useful method to diagnose and characterize PNH. This test is good for early detection of PNH clones in AA patients at initial diagnosis. PNH clone search using specific erythroid markers (CD 55 and CD59) should improve the specificity of our results.
Keyword(s): Aplastic anemia, BMT, Flow cytometry, Paroxysmal nocturnal hemoglobinuria (PNH)
Session topic: Publication Only
Type: Publication Only
Background
Flow cytometric immunophenotyping has become the gold standard for the diagnosis and monitoring of Paroxysmal hemoglobinuria(PNH). PNH results from acquired somatic mutation of the phosphatidylinositol glycan complementation class A (PIG-A) gene, leading to partial or complete absence of the glycosylphosphatidylinositol (GPI) anchor that is responsible for linking a large number of proteins to the cell membrane.
Aims
The aim of our work is to detect and quantify by flow cytometry (FC) PNH clones in aplastic anemia (AA) patients.
Methods
From January 2001 to december 2013,we have evaluated 103 patients with aplastic anemia. The presence of a PNH clone was assayed by peripheral blood flow cytometry on granulocytes(CD16) and monocytes(CD14). The diagnosis of PNH was retained by the presence of a deficit clone greater than or equal to 50% of white blood cells. The PNH clone is considered small if > 5%, and major if >30%.
Results
Among all AA patients, 15% patients had the PNH clone. Ham's test was performed in ¾ of cases. Anemia was present in all patients with PNH-AA syndrome. Among them,80% had severe aplastic anemia. Bone marrow transplantation (BMT) concened 21 % of patients with PNH-AA syndrome. three patients with PNH-AA syndrome died by severe thrombotic accident, progression to acute leukemia and severe anemia.
| Patients number | Percentage |
Major HPN clone | 5 | 5% |
Small HPN clone | 10 | 10% |
No clone HPN | 88 | 85% |
Total | 103 | 100% |
Summary
The flow cytometry on granulocytes is a useful method to diagnose and characterize PNH. This test is good for early detection of PNH clones in AA patients at initial diagnosis. PNH clone search using specific erythroid markers (CD 55 and CD59) should improve the specificity of our results.
Keyword(s): Aplastic anemia, BMT, Flow cytometry, Paroxysmal nocturnal hemoglobinuria (PNH)
Session topic: Publication Only