A Case of Pure Red Cell Aplasia Associated with Angioimmunoblastic T-cell Lymphoma
0
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Pure red cell aplasia is a bone marrow failure characterized by a progressive normocytic anemia and reticulocytopenia without leucopenia and thrombocytopenia. It is associated with various hematologic diseases. However, pure red cell aplasia with angioimmunoblastic T cell lymphoma has rarely been reported. Here we describe a 43-year-old woman with pure red cell aplasia associated with angioimmunoblastic T-cell lymphoma. She had severe anemia (hemoglobin 6.9 g/dL) and a low reticulocyte count (0.2%). Direct and indirect Coombs’ tests were positive. A CT scan of the abdomen revealed marked hepatosplenomegaly and small multiple lymphadenopathies. A bone marrow biopsy revealed focal infiltration of abnormal lymphoid cells and absence of red cell precursors. Splenic biopsy was compatible with angioimmunoblastic T-cell lymphoma. Ultimately, diagnosis of pure red cell aplasia associated with angioimmunoblastic T-cell lymphoma was made. After initiating CHOP therapy, the patient achieved complete remission, which was accompanied, shortly thereafter, by a rise in hemoglobin levels which finally returned to normal.
Abstract
Pure red cell aplasia is a bone marrow failure characterized by a progressive normocytic anemia and reticulocytopenia without leucopenia and thrombocytopenia. It is associated with various hematologic diseases. However, pure red cell aplasia with angioimmunoblastic T cell lymphoma has rarely been reported. Here we describe a 43-year-old woman with pure red cell aplasia associated with angioimmunoblastic T-cell lymphoma. She had severe anemia (hemoglobin 6.9 g/dL) and a low reticulocyte count (0.2%). Direct and indirect Coombs’ tests were positive. A CT scan of the abdomen revealed marked hepatosplenomegaly and small multiple lymphadenopathies. A bone marrow biopsy revealed focal infiltration of abnormal lymphoid cells and absence of red cell precursors. Splenic biopsy was compatible with angioimmunoblastic T-cell lymphoma. Ultimately, diagnosis of pure red cell aplasia associated with angioimmunoblastic T-cell lymphoma was made. After initiating CHOP therapy, the patient achieved complete remission, which was accompanied, shortly thereafter, by a rise in hemoglobin levels which finally returned to normal.
참고문헌 (7)
- Djaldetti M, "Pure red cell aplasia--a rare disease with multiple causes", Biomed Pharmacother, 57, pp.326~332 (2003)
- Lynch JW, "Pure red cell aplasia associated with angioimmunoblastic lymphadenopathy with dysproteinemia", Am J Hematol, 46, pp.72~78 (1994)
- Tsujimura H, "Pure red cell aplasia complicated by angioimmunoblastic T-cell lymphoma: humoral factor plays a main role in the inhibition of erythropoiesis from CD34 (+) progenitor cells", Am J Hematol, 62, pp.259~260 (1999)
- Higuchi T, "Hypocomplementemia and hematological abnormalities in immunoblastic lymphadenopathy and immunoblastic lymphadenopathylike T cell lymphoma", Acta Haematol, 96, pp.68~72 (1996)
- Alberti E, "Angioimmunoblastic Lymphadenopathy with Dysproteinemia. Report of a case with pure red cell aplasia", Haematologica, 67, pp.919~925 (1982)
- Al Hilali M, "Pure red-cell aplasia secondary to angioimmunoblastic lymphadenopathy", J R Soc Med, 76, pp.894~895 (1983)
- Dogan A, "Angioimmunoblastic T-cell lymphoma", Br J Haematol, 121, pp.681~691 (2003)