While anemia responded to intravenous (IV) methylprednisolone pulses in 2 patients (RAG1 and NHEJ1 defect each), pt.42 with STK4 defect received IV rituximab (375 mg/m2 2 doses) for control of AIHA and she did not have further relapse of AIHA for next 1.5 years. Here, NHEJ1 is linked to anemia (phenotype).