A Case Series On Plasmablastic Lymphoma Biology Essay

High grade non-Hodgkin ‘s lymphoma ( NHL ) , Kaposi ‘s sarcoma and invasive cervical malignant neoplastic disease, are the AIDS shaping malignant neoplastic diseases in HIV septic populations, although several other malignances occur with increasing frequences in HIV patients. Plasmablastic lymphoma ( PBL ) is a rare NHL discrepancy specifically associated with HIV and constitutes __ % of all HIV related lymphoma ( 1,2 ) . It expresses well-differentiated plasma cell markers like CD138, bright CD38, MUM1, alternatively of conventional B-cell markers like CD20, CD3, S100, HMB45 ( 3 ) . Plasmablastic lymphoma is reported to hold a preference for unusual sites like unwritten pit, orbit and gorge, and has hapless endurance rates ( 4 ) .

We reviewed 6 instances of plasmablastic lymphoma in HIV postitive patients who had been diagnosed and treated at the sections of Medicine and Medical Oncology, Medical College, Kolkata, India, over an 18 month period from June 2010 to December 2011. Interestingly, all 6 patients had engagement at sites other than the unwritten pit, which has been reported to be the most common site of happening of PBL ( 3 ) . We discuss the diverse clinical presentation and organ engagement by PBL in these 6 patients and their histopathological and immunohistological belongingss. We besides report on the response to standard chemotherapy and the survival rates of these survey topics on short term follow up footing.

Case series

Out of the HIV positive patients who attended the HIV Referral Clinic at Medical College, Kolkata between June 2010 to December 2011, 6 were diagnosed to be enduring from plasmablastic discrepancy of non-Hodgkin ‘s lymphoma. All of them were admitted to the Department of Medicine, where they underwent clinical scrutinies and relevant probes, including radiological and histopathological scrutinies. The assorted imaging modes were undertaken and analysed by the same radiotherapist ; likewise, the histopathogical and immunohistological slides were reviewed by the same oncopathologist, before a diagnosing of plasmablastic lymphoma was ascertained. The patients were later transferred to the section of Medical Oncology, where they received chemotherapy and supportive intervention, and reviewed on a regular basis on follow up upto 12 months.

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The mean age of the 6 patients was 50.8 ± 3.8 old ages, all of whom were male. All of them were known to be HIV positive ; the mean continuance of seropositive position being 25.3 months ( run 3.5 to 46 months ) . Mean CD4 count at presentation was 125.5 ± 71.1 cells/?L. 5 of the patients were on extremely active antiretroviral therapy ( average continuance 26.2 months ) , while one patient who had been diagnosed HIV seropositive 3.5 months ago had refused the HIV Referral Center ‘s offers for antiretroviral therapy The most common symptom at presentation was fever ( 4/6, 66.7 % ) , followed by thorax weightiness and weight loss ( 2/6, 33.3 % each ) and 1 patient each with failing of both lower limbs, surcease of micturition and oculus puffiness.

Each patient is described in brief with respects to clinical and radiological findings:

Patient 1:

47 twelvemonth old male patient who had presented with febrility, shortness of breath and right sided chest weightiness of 3 months continuance. He was found to hold lividness, lymphadenopathy and a right sided pleural based mass, from which a CT-guided biopsy was obtained.

Patient 2

46 twelvemonth old male who had increasingly diminishing urine end product over 1 hebdomad, developed azotemic symptoms after admittance along with anuresiss and steadily lifting serum creatinine. A big pelvic mass was palpated, which was confirmed on echography and was responsible for complete obstructor of both ureters, taking to bilateral hydronephrosis and clogging nephritic failure. A CT guided biopsy of the pelvic mass was obtained, followed by bilateral ureteral stenting for alleviation of obstructor. The patient succumbed to nephritic failure in malice of stenting and haemodialysis, and the biopsy findings were obtained station mortem.

Patient 3

53 twelvemonth old male with generalized lymphadenopathy who developed sudden oncoming dual vision and a quickly progressive painful proptosis in the right oculus. CT scan of his encephalon and orbit revealed a right sided retroorbital mass which was responsible for the proptosis and was besides compacting the right ocular nervus. He refused consent for a guided mulct needle aspiration to be performed on the retroorbital mass. An deletion biopsy was performed from his cervical lymph node. He refused consent for a guided mulct needle aspiration to be performed on the retroorbital mass.

Patient 4

55 twelvemonth old alcoholic agony from hurting venters, febrility and weight loss, was found to hold a big ( 10 X 8 centimeter ) suprarenal mass, which was compacting the liver every bit good as incasing the great vass. A guided trucut biopsy was obtained from the suprarenal mass.

Patient 5

50 twelvemonth old male tobacco user who presented with febrility, lividness and lymphadenopathy and who was later diagnosed to be holding a solid mass in the left lower lobe of lung, attach toing pleural gush and mediastinal lymphadenopathy.

Patient 6

54 twelvemonth old patient with history of defaulting on antiretroviral therapy presented with febrility for 4 months and a spastic paraplegia easy come oning over 2 hebdomads, along with intestine and vesica engagement. Magnetic resonance imagination of his spinal column revealed a soft tissue mass in lower thoracic part doing compressive myelopathy. A CT guided trucut biopsy was performed later.

All the biopsy specimens were subjected to histopathological and immunohistochemical surveies at the oncopathology research lab of the section of Medical Oncology, and were opined on by the same histopathologist. In all the biopsy samples obtained from different beginnings ( lymph node-1, pleural mass-1, lung mass-1, suprarenal mass-1, pelvic mass-1, paraspinal mass-1 ) , the prevailing histopathogical image was sheets of monomorphic population of big lymphoid cells with outstanding nucleoles, light to chair profoundly basophilic cytol and frequent mitotic figures, suggestive of diffuse big B-cell lymphoma. Immunohistochemical surveies revealed that the lymphoma cells in all biopsy samples phenotypically expressed the plasma cell markers MUM-1/IRF4, CD38, CD138/syndecan and were negative for B-cell markers ( CD3, CD20, CD30, CD79A, ALK-1, BCL-6, PAX-5 ) . Among other cell markers, CD56, CD10, CD4 and Bcl-2 were positive in 4 ( 66.7 % ) , 4 ( 66.7 % ) , 2 ( 33.3 ) and 1 ( 16.7 % ) patients severally. Serologic proving for presence of Epstein Barr virus was performed in all patients except patient 2 ; out of the 5 of them, 3 ( 60 % ) demonstrated the presence of IgG antibodies to Epstein Barr viral mirid bug antigen.

The 5 lasting patients were instituted with chemotherapy for lymphoma ( Regime: cyclophosphamide, doxorubicin, Oncovin, Orasone ) under the auspices of the section of Medical Oncology. All of them attained partial remittal ( intending _________ ) after ___ rhythms. Subsequently, 3 out of 5 patients were started on infusional EPOCH therapy, out of whom 2 patients attained near entire arrested development after __ months/__ rhythms of chemotherapy, while 1 patient succumbed to chemotherapy related complications ( ___________ ) . The 2 patients who opted to stay on conventional CHOP therapy were holding stable disease at the completion of 6 monthly rhythms of chemotherapy.

Discussion:

Lymphomas happening specifically in HIV-positive patients include primary gush lymphoma ( PEL ) and plasmablastic lymphoma ( PBL ) ( 1 ) . Of these, PBL occurs most normally in immature HIV infected grownup males, the most common site of happening being the unwritten pit ( 3 ) . Other rarer sites like orbit and gorge have besides been reported in assorted instance studies ( 5,6