Acute Promyelocytic Leukemia Presenting with Central Nervous System Involvement: A Report of 2 Cases
0
조회
0
다운로드
1
인용
0
참고문헌
키워드
초록
Central nervous system (CNS) involvement in acute promyelocytic leukemia (APL) is rare, and the presence of CNS symptoms at the time of diagnosis of APL is even rarer. We report 2 cases of APL presenting with CNS involvement. A 43-yr-old woman presented with easy bruising and stuporous mentality. Her complete blood count (CBC) revealed leukocytosis with increased blasts. Bone marrow (BM) analysis was carried out, and the diagnosis of APL was confirmed. This was done by cytogenetic analysis and demonstration of PML-RARα rearrangement by reverse transcriptase PCR in the BM cells. A lumbar puncture was performed to investigate the cause of her stuporous mentality, and her cerebrospinal fluid (CSF) analysis revealed 97% leukemic promyelocytes. Despite systemic and CNS therapy, she died due to septic shock by infection and rapid disease progression only 3 days after her admission. Another patient, a 3-yr-old girl, presented with easy bruising and epistaxis, and her CBC showed pancytopenia with increased blasts. BM studies confirmed APL. Quantitative PCR for PML-RARα in the BM cells revealed a PML-RARα/ABL ratio of 0.33 and CSF analysis revealed 9.5% leukemic promyelocytes (2 of 21 cells). She received induction chemotherapy and intrathecal therapy and achieved complete remission (CR) in the BM and CNS. She has been maintained in the CR status for the past 31 months. Thus, patients with APL must be evaluated for CNS involvement if any neurological symptoms are present at the time of diagnosis.
Abstract
Central nervous system (CNS) involvement in acute promyelocytic leukemia (APL) is rare, and the presence of CNS symptoms at the time of diagnosis of APL is even rarer. We report 2 cases of APL presenting with CNS involvement. A 43-yr-old woman presented with easy bruising and stuporous mentality. Her complete blood count (CBC) revealed leukocytosis with increased blasts. Bone marrow (BM) analysis was carried out, and the diagnosis of APL was confirmed. This was done by cytogenetic analysis and demonstration of PML-RARα rearrangement by reverse transcriptase PCR in the BM cells. A lumbar puncture was performed to investigate the cause of her stuporous mentality, and her cerebrospinal fluid (CSF) analysis revealed 97% leukemic promyelocytes. Despite systemic and CNS therapy, she died due to septic shock by infection and rapid disease progression only 3 days after her admission. Another patient, a 3-yr-old girl, presented with easy bruising and epistaxis, and her CBC showed pancytopenia with increased blasts. BM studies confirmed APL. Quantitative PCR for PML-RARα in the BM cells revealed a PML-RARα/ABL ratio of 0.33 and CSF analysis revealed 9.5% leukemic promyelocytes (2 of 21 cells). She received induction chemotherapy and intrathecal therapy and achieved complete remission (CR) in the BM and CNS. She has been maintained in the CR status for the past 31 months. Thus, patients with APL must be evaluated for CNS involvement if any neurological symptoms are present at the time of diagnosis.
참고문헌 (15)
- Evans GD, "Extramedullary disease in acute promyelocytic leukemia", Leuk Lymphoma, 33, pp.219~229 (1999)
- de Botton S, "Extramedullary relapse in acute promyelocytic leukemia treated with all-trans retinoic acid and chemotherapy", Leukemia, 20, pp.35~41 (2006)
- Montesinos P, "Central nervous system involvement at first relapse in patients with acute promyelocytic leukemia treated with alltrans retinoic acid and anthracycline monochemotherapy without intrathecal prophylaxis", Haematologica, 94, pp.1242~1249 (2009)
- Chow J, "Isolated central nervous system recurrence of acute promyelocytic leukemia in children", Pediatr Blood Cancer, 52, pp.11~13 (2009)
- Kanakura Y, "Acute promyelocytic leukemia with an intracerebral mass and meningeal involvement after treatment of non-Hodgkin’s lymphoma", Cancer, 59, pp.94~98 (1987)
- Pogliani EM, "Cerebral parenchymal involvement in acute promyelocytic leukemia", Haematologica, 73, pp.71~73 (1988)
- Hara T, "Detection of acute promyelocytic leukemia (APL) cells intermediately differentiated by all-trans retinoic acid in the cerebrospinal fluid: central nervous system involvement in APL", Leuk Lymphoma, 39, pp.213~215 (2000)
- Classen CF, "Long-term remission of APL with a second allogeneic BMT after CNS relapse following HLA-identical allogeneic BMT", Bone Marrow Transplant, 32, pp.843~846 (2003)
- Collins C, "Central nervous system involvement at the time of presentation in acute promyelocytic leukemia", Pediatr Blood Cancer, 54, pp.603~605 (2010)
- Gallagher RE, "Characterization of acute promyelocytic leukemia cases with PMLRAR alpha break", fusion sites in PML exon 6: identification of a subgroup with decreased in vitro responsiveness to all-trans retinoic acid, 1995, pp.86, 1540~1547
- Parmar S, "Acute promyelocytic leukaemia:a review", Expert Opin Pharmacother, 4, pp.1379~1392 (2003)
- Sanz MA, "Management of acute promyelocytic leukemia: recommendations from an expert panel on behalf of the European LeukemiaNet", Blood, 113, pp.1875~1891 (2009)
- Bae Sung Hwa, "Meningeal Relapse in a Patient with Acute Promyelocytic Leukemia: A Case Report and Review of the Literature", Journal of Korean Medical Science, 19(2), pp.311~314 (2004), 대한의학회
- Vega-Ruiz A, "Incidence of extramedullary disease in patients with acute promyelocytic leukemia: a single-institution experience", Int J Hematol, 89, pp.489~496 (2009)
- Lee HY, "Concurrent craniospinal radiotherapy and intrathecal chemotherapy in patient with acute promyelocytic leukemia second relapsed in central nervous system (CNS) following allogeneic stem cell transplantation", JOURNAL OF NEURO-ONCOLOGY, 79, pp.73~75 (2006), SPRINGER