Autoimmune hemolytic anemia in patients with SCID after T cell-depleted BM and PBSC transplantation

138

Autoimmune hemolytic anemia (AIHA) after allogeneic hematopoietic stem cell transplantation (HSCT) is still not well characterized. The aim of this study was to analyze the incidence and risk factors for the development of AIHA, as well as its prognosis and response to treatment in a series of patients undergoing allogeneic HSCT at a single institution. Between 1996 and 2004, 272 adult patients with a variety of malignant hematopoietic disorders underwent allogeneic HSCT. Direct antiglobulin testing was performed in routine pretransfusion compatibility testing or after clinical suspicion of AIHA. Twelve patients developed AIHA after HSCT at a median time of 147 days (range, 41-170). The 3-year cumulative incidence of AIHA was 4.44%. Eight cold antibodies and four warm antibodies were detected. Multivariate analysis shows that HSCT from unrelated donors (P ¼ 0.02) and the development of chronic extensive graft-versus-host disease (GVHD) (P ¼ 0.0004) were the only independent factors associated with AIHA. Two patients are still alive. AIHA was never the primary cause of death but added morbidity in patients with other concomitant complications. Patients undergoing HSCT from unrelated donors and those who develop chronic extensive GVHD are especially predisposed for this complication.

Section: Discussionmentioning

confidence: 99%

Autoimmune hemolytic anemia following allogeneic hematopoietic stem cell transplantation in adult patients

Sanz

Arriaga

Montesinos

et al. 2007

138

“…In addition to the two children who did not receive a transplant, one patient (PIN 6) died 10 months after BMT of bacterial sepsis, one patient (PIN 7) died 5.5 years post BMT of severe hemolytic uremic syndrome and chronic lung disease, and two patients (PIN 12, 15) died of autoimmune hemolytic anemia at 5 months and 7.5 years post initial BMT, respectively. 17 All four of these children were recipients of haplocompatible T cell-depleted parental grafts pre-conditioned with cyclophosphamide alone (one patient), cyclophosphamide and busulfan (one patient), or cyclophosphamide and TBI (two patients).…”

Section: Post-transplant Coursementioning

confidence: 99%

Bone marrow transplantation for T−B− severe combined immunodeficiency disease in Athabascan-speaking native Americans

O'Marcaigh

DeSantes

et al. 2001

Self Cite

Summary:A distinct form of autosomal recessive T − B − severe combined immunodeficiency disease occurs with a high frequency among Athabascan-speaking Native Americans (SCIDA), including Navajo and Apache Indians from the southwestern US and Dene Indians from the Canadian Northwest Territories. The SCIDA gene has been linked to markers on chromosome 10p although its identity and role in the pathogenesis of this disease are unknown. We report our experience in treating 18 Navajo and Dene children with SCIDA between 1984 and 1999; 16 underwent bone marrow transplants (BMT). All children were symptomatic within 2 months of birth, had the T − B − NK + SCID phenotype and 67% presented with oral and/or genital ulcers. Three children had evidence of maternal engraftment prior to transplant. Two children died shortly after diagnosis. Three children required more than one BMT and 12 are alive with T cell reconstitution at a median followup of 7 years. Three children developed normal B cell immunity, two of whom received ablative conditioning therapy with either radiation or busulfan. Three of the four children who died received therapy with either radiation or busulfan and two of eight long-term survivors who were also recipients of cytotoxic chemotherapy have failed to develop secondary teeth. These results demonstrate the efficacy of BMT in treating infants with this distinct form of SCID, although B cell reconstitution remains a problem even with HLAmatched donors. Without conditioning, T cell engraftment is likely when closely HLA-matched donors are used. With T cell depletion of haplocompatible marrow, conditioning with immunosuppressive therapy may be necessary; however, children with SCIDA who were treated with intensive immunosuppressive and

“…Like patients reported in other series, they had nonmalignant disorders, received grafts depleted of T lymphocytes, and had a late onset of AIHA. 34,35 However, neither of these patients had GvHD, and neither was receiving immunosuppressive therapy when the anemia developed. In toto, these observations suggest that TBI provides adequate immunosuppression for establishing hematopoiesis for recipients of T-cell-depleted grafts, while not increasing toxicity.…”

Section: Discussionmentioning

confidence: 99%

Allogeneic bone marrow transplantation for children with histiocytic disorders: use of TBI and omission of etoposide in the conditioning regimen

Hale

Bowman

Woodard

et al. 2003

Summary:The histiocytoses are rare disorders of antigen-processing phagocytic or antigen-presenting cells. Allogeneic bone marrow transplantation (BMT) can be curative of these disorders. We report a series of five children with Langerhans cell histiocytosis (n ¼ 2) or hemophagocytic lymphohistiocytosis (n ¼ 3), who received allogeneic BMT with a total body irradiation (TBI)-containing regimen (TBI, cytarabine, and cyclophosphamide) at our institution between 1995 and 2000. One of these patients received busulfan, cyclophosphamide, and etoposide for the first of two BMTs. All grafts except one (a matched sibling-donor graft) were T-cell-depleted grafts from unrelated donors. All received cyclosporine graft-versushost disease (GvHD) prophylaxis; the recipient of the matched sibling graft also received methotrexate. Three patients engrafted at a median of 24 days after transplantation. The patient who did not receive TBI experienced primary graft failure and recurrent disease. After the TBI-containing conditioning regimen was given, a second transplant engrafted on day +17. One patient with concurrent myelodysplastic syndrome died of toxicity on day +33 without evidence of engraftment. No acute or chronic GvHD was observed. Four patients survive disease-free, a median of 63 months after transplantation, all with Lansky performance scores of 100. We conclude that a conditioning regimen containing TBI but not etoposide is effective in allogeneic BMT for children with histiocytic diseases. Bone Marrow Transplantation (2003) 31, 981-986.