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Objectives Hematologic abnormalities are common in systemic lupus erythematosus (SLE), both at disease onset and during its progression. We present a case of severe, corticosteroid-refractory thrombocytopenia in SLE, illustrating the complexity of diagnosis and management.Methods A 35-year-old heavy-smoking woman presented with a disseminated erythematous-squamous rash, dry necrosis of all digits in both hands, and alopecia developing over four months. She reported fatigue, anorexia, and significant weight loss (~15 kg). Her history included three full-term pregnancies, with onset of cutaneous manifestations two months after the last delivery.Laboratory results showed severe anemia (Hb 6.8 g/dl) and pancytopenia (leukocytes 1400/mm3 with lymphopenia, platelets 30,000/mm3), marked inflammation (ESR 110 mm/h), and profound hypocomplementemia (low C3, C4). Mild hepatic cytolysis was noted, with normal renal function and 24-hour proteinuria. Extensive infectious screening was negative.Immunological tests revealed high-titer antinuclear antibodies, positive anti-dsDNA and anti-Sm antibodies, and circulating cryoglobulins. Imaging showed small pericardial and ascitic effusions, multiple axillary and supraclavicular lymphadenopathies, hepatomegaly, and a borderline enlarged spleen.Results A diagnosis of systemic lupus erythematosus was established, and pulse methylprednisolone with hydroxychloroquine was initiated. Three days after admission, the patient developed a brief seizure and transient topographical disorientation without brain lesions on imaging.Given the combination of digital necrosis, severe thrombocytopenia, and neurological involvement, thrombotic thrombocytopenic purpura was suspected; however, ADAMTS13 activity was normal, and antiphospholipid and antiplatelet antibodies were negative. Because thrombocytopenia worsened (18,000/mm3) and was refractory to corticosteroids, five sessions of plasma exchange and intravenous immunoglobulin were performed, leading to gradual clinical and laboratory improvement.Tissue biopsy and immunohistochemical evaluation of the necrotic digits were not available at that time, but the clinical picture was suggestive of lupus-related vasculitis or immune-mediated thrombocytopenia. The patient was discharged after three weeks and later readmitted for amputation of necrotic digits.Abstract PO:04:104 Figure 1Conclusions In SLE patients with severe, non-responsive cytopenia, collaboration with hematologists is crucial. Evaluation for reversible causes—infectious, immune, or drug-induced—is essential. Intravenous immunoglobulin, rituximab, or splenectomy may be considered in refractory cases, given the uncertain prognosis of such patients.