We report the case of a young woman who developed aplastic anemia aplastic anemia: (ay-PLASS-tik uh-NEE_mee-uh) A rare and serious condition in which the bone marrow fails to make enough blood cells - red blood cells, white blood cells, and platelets. The term aplastic is a Greek word meaning not to form. Anemia is a condition that happens when red blood cell count is low. Most… (AA), following a serologically confirmed primary Epstein–Barr virus (EBV) infection, occurring with fever and pharyngotonsillitis, in the absence of either palpable lymph nodes or enlarged spleen. Pancytopenia Pancytopenia: A shortage of all types of blood cells - red blood cells, white blood cells, and platelets. persisted after EBV DNA clearance, requiring multiple red blood cell red blood cell: The most numerous type of blood cell in healthy people. Red blood cells contain hemoglobin, a protein that picks up oxygen in the lungs and brings it to cells in all parts of the body. Also called erythrocyte, RBC. and platelet platelet: The smallest type of blood cell. Platelets help the blood to clot and stop bleeding. Also called a thrombocyte. transfusions. Given the availability of a human leukocyte antigen human leukocyte antigen: (LEW-kuh-site ANT-i-jun) One of a group of proteins found on the surface of white blood cells and other cells. These antigens differ from person to person. A human leukocyte antigen test is done before a stem cell transplant to closely match a donor and a recipient. Also called HLA. (HLA)-matched sibling donor (MSD), hematopoietic stem cell transplantation (HSCT) from bone marrow bone marrow: The soft, spongy tissue inside most bones. Blood cells are formed in the bone marrow. source was performed after a non-myeloablative conditioning regimen with cyclophosphamide cyclophosphamide: Cyclophosphamide is in a class of medications called alkylating agents. When used to treat cancer, it works by slowing or stopping the growth of cancer cells in your body. When cyclophosphamide is used to treat bone marrow failure, it works by suppressing your body's immune system. and thymoglobulin. Graft-versus-host disease (GVHD) Graft-versus-host disease (GVHD): Also called GVHD, it is a common complication of bone marrow/stem cell transplantation. It is caused when the donor's immune cells, now in the patient, begin to see the the patient's body as foreign and mount an immune response. GVHD most commonly effects the recipient's skin, intestines, or liver… prophylaxis consisted of cyclosporine cyclosporine: Cyclosporine is used along with antithymocyte globulin (ATG), another immunosuppressant, for treating aplastic anemia and some other forms of bone marrow failure. A and methotrexate. EBV reactivation occurred, one month post-HSCT, peaking at 28,838 DNA copies/ml in peripheral blood, without evidence of post-transplant lymphoproliferative disorder. Two pre-emptive doses of rituximab were administered, resulting in sustained EBV DNA negativity. Subsequent bone marrow evaluation showed normal cellularity cellularity: How much of the bone marrow volume is occupied by various types of blood cells. and restoration of peripheral counts. After two years of follow-up, the patient remains transfusion-independent, with stable hematologic recovery, no signs of GVHD, and persistent mixed chimerism (70–75% host cells in peripheral blood; about 60% donor CD3 + lymphocytes). To our knowledge, this is the only second reported case of EBV-related AA successfully treated with MSD HSCT. This case underscores the importance of assessing EBV serology in all patients with AA, since EBV infection may be mild or subclinical, and highlights the efficacy of early rituximab administration in high-level EBV DNA reactivation after transplantation.
Keywords: Allogeneic stem cell transplantation; Aplastic anemia; Bone marrow failure Bone marrow failure: A condition that occurs when the bone marrow stops making enough healthy blood cells. The most common of these rare diseases are aplastic anemia, myelodysplastic syndromes (MDS) and paroxysmal nocturnal hemoglobinuria (PNH). Bone marrow failure can be acquired (begin any time in life) or can be… ; EBV reactivation; Epstein–Barr virus; Pancytopenia.