Track: Case Reports

Abstract:

A 27-year-old male with a past medical history of severe combined immunodeficiency status-post stem cell transplant in infancy was hospitalized for workup of chronic diarrhea and chronic kidney disease. Six months earlier he had been diagnosed with a clonal T-cell large granular lymphoproliferative disorder involving bone marrow, blood, and lymph nodes. During his hospitalization, dermatology was consulted for brown to pink thin papules on the anterolateral neck, axillae, right upper arm, and left chest. The patient reported that the eruption had been present for at least one year and had been nonresponsive to high potency topical steroids. Skin biopsy from the left chest showed a brisk perivascular and interstitial nodular infiltrate of lymphocytes and admixed aggregates of histiocytes. The inflammatory infiltrate was composed of CD3-positive T-lymphocytes, the majority of which expressed CD8, TIA and granzyme. These findings were favored to be consistent with cutaneous involvement of the patient’s known T-cell large granular lymphoproliferative disorder. T-cell large granular lymphoproliferative disorder is a very rare disorder, with one registry reporting an incidence of 0.72 per 1,000,000 person-per year. Cutaneous involvement is extremely rare, with few reported cases in the literature. Given the rarity and variable clinical morphology of cutaneous involvement (eg. violaceous papules and plaques, subcutaneous nodules, necrotic plaques, etc.), accurate diagnosis may be challenging for both pathologist and clinician without a pre-existing high index of suspicion, such as in our case where the patient had known systemic disease.

Jordan Tanner

Affiliation

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(195) Cutaneous involvement of T-cell large granular lymphoproliferative disorder in a patient with a history of severe combined immunodeficiency