Acral vascular syndrome Lennert type T cell lymphoma—a case report

Abstract

Background: Acral vascular syndrome clinically presents as digital ischemia with Raynaud’s phenomenon and erythromelalgia but can be rarely seen in the malignant condition. Patients may present pain, permanent digital blanching or cyanosis, and desquamation or ulceration of the fingers. Acral vascular syndrome is rarely associated with lymphoid neoplasm and is associated with smoking, autoimmune connective tissue diseases, and vasculitis. Here, we describe a 79-year-old female who was diagnosed with vitiligo and peripheral T cell lymphoma Lennert type stage 4 with anemia of chronic disease with digital acral vascular syndrome. Case presentation: A 79-year-old female with vitiligo presented with gangrene of the distal extremities associated with pain and intermittent fever for 2 months. On evaluation, she was found to have anemia of chronic disease and generalized lymphadenopathy and diagnosed as peripheral T cell lymphoma Lennert type with bone marrow involvement and digital acral vascular syndrome. Conclusion: Acral vascular syndrome can be a presentation of lymphoma; if intervened earlier, the patient can be saved from the amputation of fingers or affected limb. Though it is a rare presentation of lymphoma, it should be considered if there is a rapid progression of gangrene. Early initiation of chemotherapy may result in the reduction of further progression of digital gangrene and thus prevents functional dependence on caregivers. In our patient, gangrene of other fingers was prevented even though it is an aggressive variant of T cell lymphoma.