A Four-year Spontaneous Remission Of Angioimmunoblastic T-cell Lymphoma

Abstract

A 66-year-old man presented with fevers, myalgia, weight loss and an urticarial rash. A full blood count showed haemoglobin concentration 70 g/l and white cell count 1·7 × 109/l with rouleaux and lymphoplasmacytoid lymphocytes seen in the blood film. He was positive for anti-neutrophil cytoplasmic antibodies (cANCA) and anti-proteinase 3 (weakly). Parvovirus immunoglobulin (Ig) M and IgG antibodies were detected, but polymerase chain reaction (PCR) for parvovirus DNA was negative. A computed tomography (CT) scan demonstrated widespread lymphadenopathy and hepatosplenomegaly (top left). A lymph node biopsy showed proliferation of capillaries with an infiltrate of small to medium sized atypical lymphocytes admixed with eosinophils, plasma cells and large blasts (bottom left). These lymphocytes were CD2 + CD3 + CD5 + with the blasts being CD10 + on immunohistochemistry, confirming a diagnosis of angioimmunoblastic T-cell lymphoma (AITL). Prior to starting treatment, the symptoms improved spontaneously with normalisation of his full blood count. Repeat CT imaging 5 months after the initial presentation confirmed complete remission (top centre). He remained well for 52 months before re-presenting with a similar symptom complex, including rash, sweats and weight loss, with widespread lymphadenopathy and splenomegaly on CT imaging (top right). Serological tests for human immunodeficiency virus (HIV) and human T-cell lymphotropic virus 1 (HTLV1) were positive, but an HIV line immunoassay and HTLV1 PCR confirmed neither infection was present. A lymph node biopsy showed increased blood vessels with a polymorphous lymphoid infiltrate, including some blasts, and numerous eosinophils (bottom right). The blasts co-expressed CD10 and PD-1 (also termed PDCD1) on immunohistochemistry, consistent with relapsed AITL. The patient was subsequently commenced on combination chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP) achieving a very good partial response after four cycles. AITL is frequently associated with abnormal immune phenomena, such as inflammatory rashes and false positive serology results. Spontaneous remissions are rare and usually short-lived, with a median of 9 months (Pangalis et al, 1983) but, as shown in our patient, can be more sustained.