PMID- 35392836 OWN - NLM STAT- MEDLINE DCOM- 20220411 LR - 20220513 IS - 1471-2369 (Electronic) IS - 1471-2369 (Linking) VI - 23 IP - 1 DP - 2022 Apr 7 TI - Acquired aplastic anemia complicated with anti-glomerular basement membrane disease successfully treated with immunosuppressive therapy: a case report. PG - 136 LID - 10.1186/s12882-022-02772-0 [doi] LID - 136 AB - BACKGROUND: Aplastic anemia (AA) is a rare but fatal disorder characterized by pancytopenia due to bone marrow hypoplasia. Anti-glomerular basement membrane disease (anti-GBM disease) is an immune complex small-vessel vasculitis that presents as rapidly progressive glomerulonephritis and/or pulmonary hemorrhage. Although both involve autoreactive T cells that are partially triggered by human leukocyte antigen (HLA)-DR15, there have been no reports of their co-existence and the treatment strategy is not well understood. CASE PRESENTATION: A 67-year-old woman presented with fever, malaise, and acute kidney injury with proteinuria and hematuria requiring hemodialysis. She was diagnosed with anti-GBM antibody disease based on high serum anti-GBM antibody titer and crescentic glomerulonephritis on a renal biopsy. Pulse administration of methylprednisolone (MP), oral prednisolone (PSL), and plasmapheresis were performed. Only 2 weeks after the diagnosis of anti-GBM disease, the patient developed pancytopenia requiring frequent blood transfusions. The blood cell count did not recover even 1 month after discontinuing the drugs that could cause pancytopenia. Bone marrow examination showed hypocellularity without abnormal infiltrates or fibrosis, which led to the diagnosis of severe acquired AA. Further HLA phenotyping revealed that she had HLA-DR15. Increased dose of PSL with the secondary MP pulse and the addition of cyclosporine improved pancytopenia. Although she remained dialysis-dependent, anti-GBM disease and pancytopenia did not recur for more than 2 years. CONCLUSIONS: We report the first case of acquired AA complicated with anti-GBM disease in an elderly woman with HLA-DR15, which was successfully treated with immunosuppressive therapy (IST). This report is valuable not only because it shows they may co-occur, but also because it provides a therapeutic option for this complex condition. It was also suggested that pancytopenia in patients with anti-GBM disease recalls serious hematologic diseases including AA that require immediate treatment based on bone marrow examination. CI - (c) 2022. The Author(s). FAU - Matsui, Kenji AU - Matsui K AUID- ORCID: 0000-0003-2897-2285 AD - Kidney Disease and Transplant Center, Shonan Kamakura General Hospital, 1370-1, Okamoto, Kamakura, Kanawaga, 247-8533, Japan. kematsui@gmail.com. FAU - Kamata, Wataru AU - Kamata W AD - Division of Hematology, Shonan Kamakura General Hospital, Kamakura, Kanawaga, Japan. FAU - Mochida, Yasuhiro AU - Mochida Y AD - Kidney Disease and Transplant Center, Shonan Kamakura General Hospital, 1370-1, Okamoto, Kamakura, Kanawaga, 247-8533, Japan. FAU - Ishioka, Kunihiro AU - Ishioka K AD - Kidney Disease and Transplant Center, Shonan Kamakura General Hospital, 1370-1, Okamoto, Kamakura, Kanawaga, 247-8533, Japan. FAU - Moriya, Hidekazu AU - Moriya H AD - Kidney Disease and Transplant Center, Shonan Kamakura General Hospital, 1370-1, Okamoto, Kamakura, Kanawaga, 247-8533, Japan. FAU - Hidaka, Sumi AU - Hidaka S AD - Kidney Disease and Transplant Center, Shonan Kamakura General Hospital, 1370-1, Okamoto, Kamakura, Kanawaga, 247-8533, Japan. FAU - Ohtake, Takayasu AU - Ohtake T AD - Kidney Disease and Transplant Center, Shonan Kamakura General Hospital, 1370-1, Okamoto, Kamakura, Kanawaga, 247-8533, Japan. FAU - Tamai, Yotaro AU - Tamai Y AD - Division of Hematology, Shonan Kamakura General Hospital, Kamakura, Kanawaga, Japan. FAU - Kobayashi, Shuzo AU - Kobayashi S AD - Kidney Disease and Transplant Center, Shonan Kamakura General Hospital, 1370-1, Okamoto, Kamakura, Kanawaga, 247-8533, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20220407 PL - England TA - BMC Nephrol JT - BMC nephrology JID - 100967793 RN - 0 (Autoantibodies) RN - 0 (Immunosuppressive Agents) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - Aged MH - *Anemia, Aplastic/complications/drug therapy MH - *Anti-Glomerular Basement Membrane Disease/complications/diagnosis/drug therapy MH - Autoantibodies MH - Female MH - *Glomerulonephritis/diagnosis MH - Humans MH - Immunosuppression Therapy MH - Immunosuppressive Agents/therapeutic use MH - Methylprednisolone/therapeutic use MH - *Pancytopenia/complications/drug therapy PMC - PMC8991481 OTO - NOTNLM OT - Anti-glomerular basement membrane disease (anti-GBM disease) OT - Aplastic anemia (AA) OT - Case report OT - Human leukocyte antigen (HLA) OT - Immunosuppressive therapy (IST) COIS- The authors have no competing interests to declare. EDAT- 2022/04/09 06:00 MHDA- 2022/04/12 06:00 PMCR- 2022/04/07 CRDT- 2022/04/08 05:27 PHST- 2022/02/18 00:00 [received] PHST- 2022/04/04 00:00 [accepted] PHST- 2022/04/08 05:27 [entrez] PHST- 2022/04/09 06:00 [pubmed] PHST- 2022/04/12 06:00 [medline] PHST- 2022/04/07 00:00 [pmc-release] AID - 10.1186/s12882-022-02772-0 [pii] AID - 2772 [pii] AID - 10.1186/s12882-022-02772-0 [doi] PST - epublish SO - BMC Nephrol. 2022 Apr 7;23(1):136. doi: 10.1186/s12882-022-02772-0.