scholarly journals 172: Post transplant autoimmune hemolytic anemia and other cytopenias are increased in young babies undergoing unrelated donor umbilical cord blood transplantation

2007 ◽  
Vol 13 (2) ◽  
pp. 64 ◽  
Author(s):  
K.M. Page ◽  
S. Wood ◽  
V. Prasad ◽  
P. Szabolcs ◽  
J. Kurtzberg
Keyword(s):  
Cord Blood ◽  
Hemolytic Anemia ◽  
Umbilical Cord ◽  
Umbilical Cord Blood ◽  
Autoimmune Hemolytic Anemia ◽  
Cord Blood Transplantation ◽  
Unrelated Donor ◽  
Umbilical Cord Blood Transplantation ◽  
Post Transplant ◽  
Blood Transplantation

Post Transplant Autoimmune Hemolytic Anemia and Other Cytopenias Are Increased in Very Young Babies after Unrelated Donor Umbilical Cord Blood Transplantation.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 1054-1054 ◽  
Author(s):  
Kristin M. Page ◽  
Adam Mendizabal ◽  
Paul Szabolcs ◽  
Susan Wood ◽  
Vinod Prasad ◽  
...  
Keyword(s):  
Cord Blood ◽  
Umbilical Cord Blood ◽  
Autoimmune Hemolytic Anemia ◽  
Cumulative Incidence ◽  
Acute Gvhd ◽  
Cord Blood Transplantation ◽  
Unrelated Donor ◽  
Umbilical Cord Blood Transplantation ◽  
Post Transplant ◽  
Blood Transplantation

Abstract Background: Unrelated donor umbilical cord blood transplantation (UCBT) is beneficial in the treatment of very young babies with early infantile, lysosomal storage diseases and hemoglobinopathies, diagnosed in utero or in the neonatal period. Methods: Over the past 9 years, we have treated 18 neonates (≤3 months of age at transplant) with Krabbe disease (n=11) metachromatic leukodystrophy (n=1), Tay Sachs disease (n=1), Hurler syndrome (n=2), Hunter syndrome (n=2), and Beta-Thalassemia Major (n=1) with UCBT after myeloablative conditioning therapy with Busulfan, Cyclophosphamide and ATG. All babies received cyclosporine + methylprednisolone (n=16) or cyclosporine + cellcept (n=2) for 9 months post transplant for prophylaxis against GvHD. Engraftment, acute and chronic GvHD, survival, treatment related mortality, and deaths were scored. Results: Eighteen babies were transplanted and 17 were evaluable for engraftment, GvHD, and survival. One baby died before engraftment of pulmonary hypertension day 15 post transplant. The cumulative incidence of overall survival was 94.4% (95% CI 83.9%–100.0%), 88.9 (95% CI 74.4%–100.0%) and 77.8% (95% CI 53.8%–100.0%) at 1, 2, and 5 years, respectively. In these infants receiving very high cell doses from their UCB graft (median total nucleated cell dose of 18.71x107/kg), neutrophil engraftment with an ANC >500/uL occurred at a median of 19 days with a cumulative incidence of engraftment of 94.1% (95%CI 77.9%–100.0%) by 42 days. Platelet engraftment (platelet count of 50K/uL untransfused) occurred in a median of 56 days with a cumulative incidence of engraftment of 94.1% (95%CI 77.8%–100.0%) at 6 months post transplant. Grade I-II acute GvHD occurred in 15/18 infants while one infant developed grade III acute GvHD of skin and gut. The cumulative incidence of grade II-III acute GvHD by day +100 was 29.4% (95%CI 6.9%–51.9%). Nine of seventeen evaluable patients developed cGvHD manifesting as autoimmune cytopenias with a cumulative incidence of 41.2% (95%CI 16.9%–65.5%) and 52.9% (95%CI 28.0–77.8%) at 1 and 2 years, respectively. In six patients, cGvHD presented as autoimmune cytopenia de novo. No graft factors were identified as being significant to development of cGvHD. All patients responded to treatment with methylprednisolone, azithroprine +/− rituximab. One patient required splenectomy. In contrast, the incidence of cGvHD in a group of otherwise similar older patients was 14.7%. Conclusion: We report an unexpected and high incidence of cGvHD manifesting primarily as autoimmune hemolytic anemia with other cytopenias, post UCBT in a population of very young babies. We hypothesize that post transplant immunosuppression interferes with the normal development of the immune system in the first year of life creating immune dysregulation and graft directed cell destruction. After lytic agents to stabilize disease, removal of chronic immunosuppressive therapy appears to facilitate recovery. Alternative strategies to prevent GvHD should be considered for this unique patient population.

Refractory Autoimmune Hemolytic Anemia Following Unrelated Cord Blood Transplantation for Acute Lymphoblastic Leukaemia: A Case Report and Review of the Literature.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 5031-5031
Author(s):  
Huilan Liu ◽  
Liangquan Geng ◽  
Juan Tong ◽  
Zuyi Wang ◽  
Zimin Sun ◽  
...  
Keyword(s):  
Case Report ◽  
Cord Blood ◽  
Hemolytic Anemia ◽  
Umbilical Cord ◽  
Umbilical Cord Blood ◽  
Autoimmune Hemolytic Anemia ◽  
Cord Blood Transplantation ◽  
Unrelated Donor ◽  
Hematopoietic Stem ◽  
Blood Transplantation

Abstract Autoimmune haemolytic anaemia (AIHA) after hematopoietic stem cell transplantation(HSCT) is a rare severe complication particularly in the paediatric population who underwent umbilical cord blood transplantation(UCBT).The pathogenesis of the post-HSCT AIHA is poorly understood, which frequently difficult to treat and overall prognosis is often poor. We present a case of a 13-year-old boy who developed AIHA 6 months after UCBT with a minor ABO blood group incompatiblity, 5/6 HLA matching umbilical cord blood unit from an unrelated donor was used for high risk acute lymphocyte leukemia in third remmision. Following conditioning with busulfan, cyclophosphamide, and antithymocyte globulin (ATG), the patient underwent transplantation and attained sustained hemopoietic reconstitution without severe graft-versus-host disease. At 6 months following UCBT,he developed severe acute autoimmune hemolytic anemia associated with a “warm” IgG antibody. He was being treated with packed RBC transfusions, cyclosporine, steroids and immunoglobulins, His AIHA was partly resolved. Hemolysis recurred after steroid tapering and partially responded to rituximab (375 mg/m(2) once weekly ×4 and plasma exchange.Despite a range of complex treatment regimen, the unstable hemolytic anemia was present and repeated severe infectious episodes occurred.We reduced suppression,strengthened antibiotic therapy,Pulmonary infection and hemolysis could not be controlled, the patient died of respiratory failure and circulatory collapse finally. This case report highlights the difficulty in managing posttransplant AIHA,Further studies about the pathogenesis of AIHA associated in HSCT recipients are needed.

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