Results(Case Description)
A case of a 15-year-old female patient with warm type AIHA and TKS was previously reported.5 Besides anaemia, she exhibited severe developmental delay, dysmorphic facial features, deafness, camptodactyly, and chronic macrothrombocytopenia (60 × 103/μL). A computed tomography (CT) scan showed splenomegaly (160 mm craniocaudally).
AIHA was initially improved with intravenous prednisolone (2.0 mg/kg/day) for 24 days, followed by oral prednisolone, as previously described.5 Anaemia improved, and the dose was reduced gradually. However, AIHA relapsed when the dose of oral prednisolone was reduced to 0.19 mg/kg every other day, requiring treatment with high-dose prednisolone. AIHA relapsed every time the dose was reduced, and gradually the required amount of prednisolone increased. After anaemia became uncontrollable with prednisolone, rituximab (375 mg/m2/week, four times) was added. She had a transient response to rituximab, but again, relapsed during prednisolone tapering.
AIHA was resistant to prednisolone and rituximab, and over time, she required frequent red blood cell transfusions. In addition, the follow-up CT scan showed an exacerbation in splenomegaly (190 mm craniocaudally). Uncontrolled AIHA worsened the anaemia, which led to hypersplenism. In turn, hypersplenism worsened the anaemia (5.4 g/dL at the lowest) and thrombocytopenia (26 × 103/μL at the lowest), and enhanced autoantibody production, making AIHA even more uncontrollable (Figure 1 ). The vicious cycle resulted in the anaemia becoming drug-resistant, and the thrombocytopenia to gradually exacerbate. As her general condition worsened because of the edema and thoracicoabdominal fluid production caused by both pathologies, we decided to perform splenectomy to treat the refractory AIHA.
Post-operatively, the haemoglobin gradually normalized to 11–15 g/dL and we were able to taper prednisolone (Figure 1 ). She no longer required red blood cell transfusion or hospitalization even after discontinuation of prednisolone, although direct antiglobulin test results remained positive. Platelet counts also normalized to 230–400 × 103/μL (Figure 1 ), and the numbers of large platelets (< 8 μm) increased.