Daratumumab in severe Evans syndrome: a case report 2026 Hillmann et al.

[Jaybee00]

Evans syndrome is a rare autoimmune disorder characterized by the simultaneous or sequential occurrence of autoimmune cytopenia. The disease is chronic, relapsing, and frequently refractory to standard therapies. Typical symptoms include anemia-related fatigue, pallor, and jaundice due to hemolysis and petechiae, and purpura and mucosal bleeding due to thrombocytopenia. Treatment often involves a stepwise escalation of immunosuppressive treatments (e.g., corticosteroids, anti-CD20 monoclonal antibodies) and/or stimulants (e.g., thrombopoietin receptor agonists). However, sustained remission remains elusive in many patients. We report the case of a 69-year-old woman with a 19-year history of Evans syndrome, presenting with a life-threatening relapse marked by severe thrombocytopenia and strong hemolytic activity. The patient had previously undergone multiple treatment regimens and had developed comorbidities that both complicated disease management and treatment strategies. Despite repeated therapeutic interventions with various immunosuppressant agents, she remained transfusion-dependent and clinically unstable and experienced various treatment complications. Additionally, targeting antibody-producing plasma cells with daratumumab (anti-CD38) led to a rapid fall in transfusion dependency, clinical stabilization, and transition to outpatient care. Unfortunately, the patient later succumbed to infectious complications after a femoral fracture. This case underscores the therapeutic complexity of multirefractory Evans syndrome and the limitations of conventional therapy. The addition of daratumumab, resulting in depletion of CD38+plasma cells, helped achieve hematologic stabilization in this refractory case.

Frontiers | Daratumumab in severe Evans syndrome: a case report

Evans syndrome is a rare autoimmune disorder characterized by the simultaneous or sequential occurrence of autoimmune cytopenia. The disease is chronic, rela...

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[Jonathan Edwards]

The addition of daratumumab, resulting in depletion of CD38+plasma cells, helped achieve hematologic stabilization in this refractory case.

But may have caused her to succumb to infection.

 

[Utsikt]

But may have caused her to succumb to infection.

This is the timeline with Dara:

She presented to our outpatient clinic in January 2024 for follow-up laboratory work after a recent hospitalization in December 2023 for severe ITP.

Therefore, we decided to start therapy with daratumumab in September 2024 in an inpatient setting with 1,800 mg subcutaneously once weekly for 8 weeks.

This quickly led to a further decrease in transfusion dependency (initially just one more RBC transfusion after the sixth daratumumab injection), which also enabled treatment to be moved to an outpatient setting. This improvement most likely reflected a cumulative effect of the concurrent therapies in addition to the plasma cell depletion and immunomodulatory effect of daratumumab.

We subsequently discontinued therapy with fostamatinib, MMF, and finally, tacrolimus. The patient regained walker-assisted mobility and was able to stay in outpatient care (ECOG 2). However, her performance status remained reduced compared to her baseline at admission in January 2024 (ECOG 0), underscoring the substantial toll the disease and its treatment had taken on her overall condition.

After completing seven of eight planned subcutaneous daratumumab injections, the patient experienced a proximal femur fracture at the beginning of November, requiring surgery and postoperative RBC transfusions. Approximately 2 weeks later, she presented to the emergency room with severe pneumonia and expired within 24 h.