Wing Kit Lam¹, Victor Wai Kwan Lee¹, Shun Yin Kong², Tsz Fung Wong¹, Sze Fai Yip¹

¹Department of Clinical Pathology, Tuen Mun Hospital; ²Department of Medicine and Geriatrics, Pok Oi Hospital, Hong Kong

A 30-year-old man was admitted for on-and-off fever for 2 weeks and painful nodules over trunk and limbs for 4 months. He had a previous history of painful nodules occurred for months and subsided spontaneously but he did not seek medical attention. Physical examination showed multiple tender erythematous nodular swellings over chest wall and bilateral thighs with no lymphadenopathy. Ultrasound abdomen showed hepatosplenomegaly. He had mildly elevated ALT of 120 U/L, markedly elevated lactate dehydrogenase of 1,194 U/L and ferritin of 26,625 pmol/L. He had worsening anaemia and neutropenia, and bone marrow examination showed haemophagocytosis with no obvious marrow infiltration. Skin biopsy confirmed the diagnosis of subcutaneous panniculitis-like T-cell lymphoma (SPTCL). Homozygous HAVCR2 p.Y82C mutation (NM_032782.5:c.245A>G) was detected upon targeted Sanger sequencing. The patient was given cyclosporin A 100 mg BD PO and prednisolone 30 mg BD PO. The skin lesions gradually improve and steroid was tailed down gradually. The skin lesions resolved completely 3-month post-treatment.

Haemophagocytic lymphohistiocytosis in SPTCL is associated with adverse prognosis. There is no standard treatment approach for SPTCL. Common treatment options would include immunosuppressive therapy (steroid plus cyclosporin A) and chemotherapy followed by haematopoietic stem cell transplantation.

Author Contribution Statement: Lam WK conceptualised the study and drafted the abstract; Lam WK, Lee VWK, Kong SY, Wong TF, Yip SF collected and analysed the data. All authors reviewed and approved the abstract.