This case represents a successful example of managing hemolytic disease of the fetus due to a rare antibody using maternal blood. It also supports previous data on safety of maternal donations during pregnancy and the use of combination of rHu-EPO and IV-Fe as a supportive measure.

Familial hemophagocytic lymphohistiocytosis (FHLH) is a potentially fatal disorder of immune regulation. Management includes chemotherapy followed by hematopoietic stem cell transplantation (HSCT). T cell depleted (TCD)-haploidentical HSCT could be an option for those patients who do not have HLA matching family donor. The objective of this study was to report on the outcome of TCD-haploidentical HSCT in patients with FHLH who underwent transplantation at Sultan Qaboos University Hospital (SQUH). This is a retrospective report on 12 patients with FHLH who received TCD-haploidentical HSCT at SQUH between August 2010 and December 2018. Epidemiologic characteristics and details on the transplantation procedures and complications were collected from patients' electronic records. Twelve patients with FHLH received TCD-haploidentical HSCT after a myeloablative conditioning regimen composed of treosulfan/thiotepa/fludarabine/anti-thymocyte globulin and rituximab. The mean age at transplantation was 11.67 § 8 months. All patients had Perforin gene mutations, except 1 patient who had an UNC-13D mutation. Most patients received TCRab + /CD19 + depleted grafts for faster immune reconstitution. Seven patients (58.3%) have been cured with a mean follow-up duration of 3.44 years. Four patients died of multiorgan failure secondary to gram-negative sepsis. One patient had primary graft failure, and 2 patients had mild graft-versus-host disease. Two patients had Pneumocystis carinii pneumonia, 2 had adenoviremia, and 9 patients had cytomegalovirus (CMV) viremia. Among patients with CMV viremia, 2 had evidence of disease (retinitis, enteritis). All patients with CMV viremia were treated successfully with foscarnet pre-engraftment and ganciclovir postengraftment, respectively. TCD-haploidentical HSCT could be a viable option for patients with FHLH who do not have HLA matching family donors. Infectious complications are the leading cause of death in that setting. CMV viremia was the most frequently encountered infectious complication.

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Hemolytic disease of the fetus and newborn (HDFN) due to anti-D antibodies is a well-known complication of rhesus (Rh) incompatibility, encountered in D-positive babies born to alloimmunized D-negative mothers who have been sensitized during previous labor or abortion. Here, we report a case of significant hemolytic disease of the newborn due to the presence of anti-D antibodies in an Rh-positive baby born to an Rh-positive mother. The boy presented at day 1 of life with neonatal jaundice and required intensive phototherapy. His hemoglobin (Hb) concentration gradually dropped from 17 g/dL to 6.6 g/dL. The blood bank workup revealed O Rh-positive blood group, with a positive direct antiglobulin test (DAT) and confirmed the presence of anti-D antibodies. His mother was typed as O Rh positive, with a negative DAT and positive anti-D. He required two blood transfusions, and his Hb stabilized at the age of 7 weeks. Anti-D HDFN is a rare complication of Rh-positive and Rh-weak positive pregnancies. The lack of awareness of this phenomenon is often a source of confusion for clinicians. A literature review of similar cases and possible explanations are discussed.