Multiple Remissions of Extracavitary Primary Effusion Lymphoma Treated With a Single Cycle of Liposomal Doxorubicin in a Patient Infected With HIV

Abstract

Primary effusion lymphoma (pel) is a rare human herpesvirus 8 (hhv8)–related large B cell lymphoma with plasmablastic, immunoblastic, or anaplastic features that often carries a poor prognosis. This lymphoma occurs mainly in patients with hiv infection, most often with Epstein–Barr virus (ebv) co-infection, and usually presents as body cavity effusions or, less commonly, as extracavitary lesions without effusion (ec-pel). Chemotherapeutic treatment options are limited and require concurrent antiretroviral therapy (art).Here, we report the case of an adult patient with hiv infection and chronic hepatitis E virus (hev) co-infection who had low CD4 T cell recovery after years of art. The patient then developed a cutaneous ec-pel which rapidly regressed after 1 cycle of liposomal doxorubicin (ld) for his Kaposi sarcoma (ks) before treatment with chop chemotherapy. He had previously received numerous cycles of ld for cutaneous ks over 2 years.Because of the patient’s low CD4 T cell count, hev co-infection, and earlier unexpected remission of ec-pel before chop, the patient opted for a single trial of ld before other options. Surprisingly, he experienced a complete remission lasting 18 months. Subsequently, his ec-pel relapsed twice at 31 and at 41 months after the initial diagnosis. Upon recurrence, a similar single cycle of ld was given, which again induced remission. The patient today is in complete remission after a total of 4 ld infusions over 54 months.This patient represents a unique case of hiv-with-hhv8–related, ebv-negative ec-pel with chronic hev coinfection, in which rapid remission was achieved after a single cycle of ld, suggesting an antiviral response in addition to the chemotherapeutic effect.

Extracavitary primary effusion lymphoma recurring with syphilis in an HIV-infected patient

“…B cell receptor (BCR) gene rearrangement testing using PCR confirmed the clonality of the atypical B cells. These findings were similar to those from previous biopsies of the known EC-PEL in this patient, 2 thus consistent with rectal lymphoma relapse. He received doses of LD on 10 January and 7 February 2020 ( table 1 ) and achieved remission of EC-PEL supported by rectosigmoidoscopy examination and PET scan ( figure 2 ).…”

“…He was later diagnosed with cutaneous Kaposi sarcoma (KS) with bilateral inguinal lymphadenopathy in 2012 and with HHV8-positive but EBV-negative EC-PEL with cutaneous involvement in 2014. 2 He was treated with liposomal doxorubicin (LD) for his KS and with four cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin hydrochloride, oncovin and prednisone) for his lymphoma, as previously described. 2 His CD4 T cell count remained below 100×10 6 /L on the same antiretroviral therapy (ART), and although he had recurrences of his lymphoma (2015–2017) involving the skin and tonsils without lymphadenopathy he had been in remission since October 2017.…”

“…The patient received four cycles of R-CHOP for the previous occurrence of his lymphoma and was treated with LD for cutaneous KS lesions with successful remission. 2 Later recurrences of his EC-PEL in the skin and tonsils were treated with LD only, with unexpected remissions. 2 At the patient’s request and to avoid second-line chemotherapy, LD was thus given between 2014 and 2017 for three recurrences of EC-PEL.…”

“… 10 11 PEL typically presents as a malignant effusion in the pleural, pericardial or peritoneal cavities. 3 However, in 5% of cases, 2 lymphomas otherwise indistinguishable from PEL presenting as solid tumour masses outside body cavities have been recognised by the WHO revised fourth edition as the extracavitary variant of PEL (EC-PEL). 12 EC-PEL most commonly presents in the lymph nodes and skin 3 13 ; however, cases of gastrointestinal involvement 5 9 14–17 have been reported and their outcome seems to be better than the effusion variant.…”

A Rare Case of Extracavitary Primary Effusion Lymphoma in the Bladder and Ureter

“…PEL is most commonly seen in immunocompromised patients, such as patients with concomitant HIV infection, organ transplant, etc. As in other lymphomas, active treatment of the HIV infection, reversal of the immune suppression, takes precedence along with antilymphoma treatment as demonstrated in a recent case study of prolonged benefit of liposomal doxorubicin in an HIV infected PEL patient [18]. PEL can, however, rarely present in patients with an intact immune function, like in our patient.…”