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.