Age 1 years, Juvenile Myelomonocytic Leukemia
Contributed By: Dr. Imtenan Sharif
Husnain was just one year old when, in March 2013, he presented with history of recurrent fever and rash. He had gross hepatosplenomegaly 9 and WBC count of 40 x 10 /l with absolute monocyte count of 11.2 x 9 10 /l. There were 4% blasts on peripheral film and 8% on bone marrow examination, consistent with diagnosis of Juvenile Myelomonocytic Leukemia. PCR for BCR-ABL, Hb electrophoresis and cytogenetics were unremarkable.
Husnain reported at AFBMTC in Jun 2013. On examination liver and spleen were palpable 5 cm and 4 cm below costal margin, respectively. He was put on hydroxyurea and folic acid. The patient had HLA matched sister and was scheduled to undergo allogeneic stem cell transplant in Feb 2014.
However, the patient continued to have low grade intermittent fever. A repeat bone marrow examination demonstrated no progression of disease. Work up for infectious disease etiology also came up negative. Fever did not respond to antibiotics and antifungal therapy and was considered due to disease burden.
Therefore, before transplant, 2 courses of low dose cytarabine (100mg x 5 days) were given to which the patient responded quite well and his fever subsided. However, hepatosplenomegaly persisted (Liver 9 cm; Spleen 10 cm).
During transplant conditioning was 16 160 140 given with Bu Cy Mel (recommended by EBMT/EWOG- MDS group for children). Husnain tolerated conditioning well. Bone marrow was harvested from his 8/8 HLA matched sister on 16 May 2014. As there was an ABO mismatch, bone marrow harvest was processed which yielded a dose 8 (MNC) of 5.0 x10 /kg. The bone marrow was transfused the same day through central venous line.
Graft versus host disease (GvHD) prophylaxis was given with cyclosporin (CSA) short methotrexate (MTX). Neutrophil engraftment was achieved on D+14 and platelet engraftment later on D+87. Liver at the start of conditioning was 9 cm BCM which regressed to 5 cm at the time of discharge on D+20.
Husnain’s donor chimerism was more than 90% at 1 and 3 months. However, he was not maintaining Hb and was on regular RCC transfusions. His bone marrow examination on D+138 was consistent with post-transplant pure red cell aplasia.
The patient was given 2 doses of 2 Rituximab at 375mg/m after which his Hb came up and he became transfusion free. The donor chimerism remained more than 90% and lymphocyte subset analysis demonstrated good immune reconstitution. Immunosuppression was stopped at 6 months post-transplant. By this time there was no visceromegaly. Vaccination was carried out as per protocol.
Husnain is now 5 ½ years old and enjoys good quality of life with his family.