Effusion-associated anaplastic large cell lymphoma of the breast: time for it to be defined as a distinct clinico-pathological entity.

s/2010/4.cgi 13. Cohen PL, Brooks JJ. Lymphomas of the breast. A clinicopathologic and immunohistochemical study of primary and secondary cases. Cancer. 1991;67(5):1359-69. Highly active antiretroviral therapy alone may be an effective treatment for HIV-associated multicentric Castleman’s disease Multicentric Castleman’s disease (MCD) is a rare lymphoproliferative disorder mainly seen in HIV-infected patients and associated with poor prognosis. Pre-HAART mortality was 70-85%. Proliferation of polyclonal but often monotypic plasmablastic cells is thought to be driven by Kaposi sarcoma herpes virus (KSHV) infection, present in all patients with HIV-associated MCD. There is no standard treatment. Interventions include rituximab, lymphoma-type treatment with chemotherapy and splenectomy, Many are used concomitantly making it difficult to ascertain which is of greatest efficacy. We describe 4 cases of biopsy-proven HIV-associated MCD who showed a complete clinical and radiological response to MCD, and a reduction in KSHV viral load to HAART alone without additional therapeutic interventions. All patients are alive and relapse free 19-38 months later. Between February 1 2007 and October 1 2008, we identified 4 consecutive patients who received HAART alone for lymph node biopsy-proven HIV associated-MCD (Table 1). Median duration of HIV infection prior to MCD was two years (range 1-14). In all patients, nodes showed a mixture of reactive lymphoid follicles and follicles with irregular, regressed germinal centers. At the periphery of these regressed germinal centers were large plasmablastic cells with prominent nucleoli and basophilic cytoplasm, usually CD20 negative, and showing positive staining for KSHV, IgM and lambda light chains (Figure 1). All patients presented with fever, lymphadenopathy, hepato splenomegaly and elevated C-reactive protein (CRP). Three patients investigated with FDG PET/CT scan showed FDG avid lymphadenopathy above and below the diaphragm. Three patients had started HAART less than 35 weeks before presentation with MCD; HAART had been initiated due to a combination of CD4 decline and constitutional symptoms. HAART was modified in one patient and interrupted in one patient due to renal and liver impairment. In all patients, resolution of constitutional symptoms occurred within three months of starting HAART. Patient 2 had a recrudescence of symptoms (fever, splenomegaly and elevated CRP) eight months after diagnosis of MCD which resolved spontaneously. Serum KSHV was 6,600 copies/mL during this flare. Three months previously KSHV had been 3,600 copies/mL. Three patients who initially demonstrated significant FDG avid lymphadenopathy now showed complete metabolic response on repeat PET/CT scan and the remaining patient CRu on repeat whole body CT scan. At time of MCD diagnosis, all samples from patients were positive for KSHV between 420 and 120,000 copies/mL; 3 patients had sustained undetectable KSHV viral loads following antiretroviral therapy. KSHV was detectable in 2 patients in whom retrospective sampling of stored blood was availhaematologica | 2010; 95(11) 1979 Letters to the Editor Figure 1. Composite histological figure showing features of MCD in lymph node biopsy. (A) Routine Hematoxylin and Eosin (H&E) section of a needle core biopsy of node showing an abnormal germinal center with irregular outline and scattered large plasmablastic cells (x110 original magnification). (B) High power H&E from a whole node biopsy showing a characteristic regressed germinal center with plasmablastic cells (x220 original magnification). (C) KSHV immunohistochemistry showing a target-like arrangement of KSHV-positive plasmablasts around a germinal center. (D) Lambda light chain immunohistochemistry showing cytoplasmic positivity in the plasmablastic cells in a germinal center. A B C D