Diffuse alveolar hemorrhage in a child with mild hemophilia A who underwent bone marrow transplantation for thalassemia: A Case Report Hoa Thi Kim Nguyen, Lawrence Faulkner, Son Binh Bao Bui, Henrique Manoel Lederman, Nhan Thi Thanh Ho, Tam Thi Dang

HOA THI KIM NGUYEN^1*Lawrence Faulkner²Son Binh Bao Bui³Henrique Manoel Lederman⁴Nhan Thi Thanh Ho¹Tam Thi Dang¹

• ¹Hue Central Hospital, Hue, Vietnam
• ²Cure2Children Foundation, Florence-Italy, Florence, Italy
• ³Hue University, Hue, Vietnam
• ⁴Universidade Federal de Sao Paulo, São Paulo, Brazil

ABSTRACT Diffuse alveolar hemorrhage (DAH) is a relatively rare complication of bone marrow transplantation (BMT) associated with a high risk of mortality. It generally occurs in the early post-transplant phase during severe thrombocytopenia; however, since most thrombocytopenic cases do not develop DAH, cofactors are at play. Here, we discuss the case of a 4-year-old male child with mild asymptomatic hemophilia A discovered on routine evaluation pre-BMT, who developed DAH after a fully matched sibling BMT for HbE/Beta-thalassemia. The patient presented on day +21 post-BMT with sudden onset of cough followed one day later by hemoptysis. He had received FVIII supplementation with PTT normalization and FVIII levels of 28%. A chest CT scan showed interstitial lung disease with areas of ground glass bilaterally, mainly in the lower lobes. A radiological diagnosis of DAH was made, and the child was treated with additional FVIII concentrate (Advate), blow-by oxygen supplementation, red blood cells transfusion, platelet transfusion, methylprednisolone, tranexamic acid, and antioxidants (vitamin C and E). He responded well and recovered in a few days. Conclusion: Mild hemophilia associated with DAH post-BMT has never been reported before, however, it should be considered as a potentially treatable cause of DAH after BMT. FVIII levels should be part of the routine workup of children developing DAH post-transplantation. In the presence of thrombocytopenia high FVIII levels of >30-40% might be required to prevent/treat DAH.