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Plasmablastic Lymphoma

PBL


Presentation

  • In our first case, a 40 year-old human immunodeficiency virus positive male presented with a hemorrhoid-like sensation, and was diagnosed with PBL via biopsy of a rectal mass.[ncbi.nlm.nih.gov]
  • METHODS: The medical records were reviewed for clinical presentation, staging, laboratory data, radiological studies, treatments, and outcomes.[ncbi.nlm.nih.gov]
  • To retrospectively analyze the presentation, clinical findings, and outcome of patients presenting to our institute with a diagnosis of PBL involving the GI tract.[ncbi.nlm.nih.gov]
  • We report a case of PBL presenting as multiple violaceous nodules and plaques on the leg of a HIV-negative patient, 13-years post-renal transplant. To date, 20 cases of PBL presenting in the skin have been reported.[ncbi.nlm.nih.gov]
  • This case is unusual in that the patient presented with a large breast mass and her recovery was extremely rapid.[ncbi.nlm.nih.gov]
Generalized Lymphadenopathy
  • CASE PRESENTATION: A 19-year old woman was admitted to the hospital with generalized lymphadenopathy, a pelvic tumor mass measuring 35   30 cm and a 4 cm lump in her right breast.[ncbi.nlm.nih.gov]
  • On examination, he was emaciated and had generalized lymphadenopathy. Cutaneous examination revealed a 8 cm 6 cm 7 cm ulcero-proliferative growth in the perineal region.[ijdvl.com]
Ascitic Fluid
  • We report a case of extraoral/mesenteric PL detected using cytological examination of ascitic fluid assisted by flow cytometric (FC) analysis.[ncbi.nlm.nih.gov]
  • Extraoral plasmablastic lymphoma detected using ascitic fluid cytology and flow cytometry: a case report with a review of the literature. Rezk SA, Zhao X, Weiss LM (June 2018).[en.wikipedia.org]
Thyroid Nodule
  • He also underwent left thyroidectomy for a thyroid nodule in January 2006, which revealed a limited-stage of low-grade extranodal marginal zone lymphoma (MZL) requiring no additional treatment.[doi.org]
Rectal Pain
  • We describe a patient with acquired immunodeficiency syndrome who presented with rectal pain and bright red blood per rectum. He was diagnosed with stage IVA PBL with anorectal, nodal, calvarial and hepatic involvement.[ncbi.nlm.nih.gov]
Rectal Mass
  • In our first case, a 40 year-old human immunodeficiency virus positive male presented with a hemorrhoid-like sensation, and was diagnosed with PBL via biopsy of a rectal mass.[ncbi.nlm.nih.gov]
Fecal Incontinence
  • We report a case of plasmablastic lymphoma in a 46-year-old woman for whom the initial clinical manifestation was a painless perineal tumor accompanied by fecal incontinence.[ncbi.nlm.nih.gov]
Tenesmus
  • […] intestine ND Diarrhea, distention 1 F NL NL DOD before diagnosis made 8 Pruneri et al [ 10 ] 1998 53/F Stomach - Postprandial fullness, stomach ache F ProMACE-cytaBOM Remission Alive at 19 mo 9 Rajagopal et al [ 9 ] 2006 35/M Ano-rectal Rectal bleeding, tenesmus[doi.org]
Lesion of the Tongue
  • Herein, we report a case of PBL presenting as an ulcerated lesion of the tongue in an HIV-negative patient, 6 years after renal transplantation.[ncbi.nlm.nih.gov]
Breast Mass
  • This case is unusual in that the patient presented with a large breast mass and her recovery was extremely rapid.[ncbi.nlm.nih.gov]
Large Breast
  • This case is unusual in that the patient presented with a large breast mass and her recovery was extremely rapid.[ncbi.nlm.nih.gov]
Vaginal Bleeding
  • Herein, we report an uncommon case of plasmablastic lymphoma in a 61-year-old female with human immunodeficiency virus that presents with vaginal bleeding.[ncbi.nlm.nih.gov]
Meningism
  • This is the first reported case of PBL involving the skull and meninges in a HIV-infected child.[content.iospress.com]
  • […] his consciousness, then awoke after 4 to 5 minutes, with symptoms of angulus oris numbness and the right upper limb powerless ten days ago.MRI of the brain revealed a well-defined hyperdense and enhancing mass in the left frontal-parietal lobe, the meninges[ncbi.nlm.nih.gov]
  • The organs primarily involved were the terminal ileum, stomach, oral cavity, tonsil, nasal cavity and meninges. The tumors were histologically typical of PBL.[tandfonline.com]
Convulsions
  • China on March 9th, 2011, with chief complaints of right limbs convulsioned suddenly, then fell down and lose of his consciousness, then awoke after 4 to 5 minutes, with symptoms of angulus oris numbness and the right upper limb powerless ten days ago.MRI[ncbi.nlm.nih.gov]

Workup

  • After a staging workup including radiographic skeletal survey, positron emission tomography/computed tomography, and bone marrow biopsy, there was no evidence of other organ involvement, and serum and urine protein electrophoresis were normal.[indianjurol.com]
  • Workup of the anemia was most consistent with anemia of chronic disease. After 18 months, lenalidomide and dexamethasone were stopped. Imaging at 24 months continued to show complete remission.[karger.com]
  • Complete workup of patients including hemogram; biochemistry including lactate dehydrogenase, bone marrow biopsy, and cerebrospinal fluid examination; computed tomography (CT) scans of neck, chest, abdomen, and pelvis was done.[indianjcancer.com]
Normocytic Anemia
  • A complete blood count and basic metabolic panel were remarkable for a normocytic anemia with hemoglobin concentration of 9.9 g/dl.[karger.com]
  • He had mild normocytic anemia and normal absolute neutrophil count.[doi.org]

Treatment

  • The baseline clinical characteristics, treatment modalities, overall outcomes, and treatment-related toxicity were assessed. RESULTS: The median age at diagnosis was 50.5 years.[ncbi.nlm.nih.gov]
  • Clinicians often face the question of second or third line treatment. As the treatment options with novel agents in lymphomas are rapidly evolving, more and more options beyond standard chemotherapy are available.[ncbi.nlm.nih.gov]
  • As a distinct type of aggressive mature large B-cell lymphoma, plasmablastic lymphoma (PBL) poses diagnostic and treatment challenges.[ncbi.nlm.nih.gov]
  • PBL carries a particularly poor prognosis and more intensive treatment is usually warranted. However, no treatment guidelines exist and treatment choices are made based on case reports and small retrospective case series.[ncbi.nlm.nih.gov]
  • Our approach was based on the full application of a standard multiple myeloma treatment and, to the best of our knowledge, it represents the only reported experience so far. This treatment was overall well tolerated.[ncbi.nlm.nih.gov]

Prognosis

  • PBL represents a diagnostic challenge given its characteristic morphology and lack of CD20 expression, and also a therapeutic challenge, with early responses to therapy, but with high relapse rates and poor prognosis.[ncbi.nlm.nih.gov]
  • Host immune status and chemosensitivity are associated with improved prognosis in PBL.[ncbi.nlm.nih.gov]
  • Plasmablastic lymphoma (PBL) is a rare distinct subtype of aggressive diffuse large B-cell lymphoma and a notoriously hard to treat entity with a dismal prognosis in both HIV-negative and HIV-positive patients.[ncbi.nlm.nih.gov]
  • Plasmablastic lymphoma is a subtype of B-cell lymphoma with an aggressive behavior and a poor prognosis. C-methionine appears to be a promising tracer for paraprotein-producing neoplasms.[ncbi.nlm.nih.gov]
  • Despite intensive chemotherapy regimens and combinational antiretroviral therapy, the prognosis of PBL in HIV-infected patients remains poor.[ncbi.nlm.nih.gov]

Etiology

  • Although its exact etiology is poorly understood, Epstein-Barr virus infection and MYC gene aberrations have been implicated in its development in both HIV-positive and HIV-negative patients.[ncbi.nlm.nih.gov]
  • Abstract Castleman disease (CD) is a lymphoproliferative disorder of unknown etiology that is associated with the development of secondary tumors, including B-cell lymphoma.[ncbi.nlm.nih.gov]
  • (Etiology) Currently, the cause of Plasmablastic Lymphoma (PBL) is unknown.[dovemed.com]
  • […] available on PBL are from case reports and case series owing to the low incidence. [8] Furthermore, different populations might generate different outcomes. [8,12] In the present study, we systematically analyzed the demographic, clinicopathological, etiological[journals.lww.com]
  • However, there is little consensus concerning many aspects such as etiology, clinical findings, optimal treatment strategy and prognostic factors of HIV-negative PBL.[spandidos-publications.com]

Epidemiology

  • The objectives of this review are to summarize the current knowledge on the epidemiology, biology, clinical and pathological characteristics, differential diagnosis, therapy, prognostic factors, outcomes, and potential novel therapeutic approaches in[ncbi.nlm.nih.gov]
  • The main goal of this article is to systematically review the most recent advances in epidemiology; pathophysiology; clinical, pathologic, and molecular characteristics; therapy; and prognosis in patients with PBL.[ncbi.nlm.nih.gov]
  • CONCLUSION: Because of the consistent epidemiological association of PBL with immunosuppression, any patient diagnosed with PBL should be tested for HIV.[ncbi.nlm.nih.gov]
  • Epidemiology of AIDS-defining Malignancies William A. Blattner and Rebecca G. Nowak 3. Epidemiology of non-AIDS Defining Malignancies Andrew E. Grulich 4. HIV Cancers in Resource-Limited Regions Sam M. Mbulaiteye 5.[books.google.de]
  • Fever (for no known reason) Pain in the chest, abdomen, or bone s (for no known reason) Skin rash or itchy skin Weight loss (for no known reason) Diagnostic Exams Flow cytometry Immunohistochemistry Immunophenotyping Progression and Transformation None Epidemiology[seer.cancer.gov]
Sex distribution
Age distribution

Pathophysiology

  • The main goal of this article is to systematically review the most recent advances in epidemiology; pathophysiology; clinical, pathologic, and molecular characteristics; therapy; and prognosis in patients with PBL.[ncbi.nlm.nih.gov]
  • Pathophysiology The pathophysiology is incompletely understood, but the plasmablast is known to be the cell of origin.[ncbi.nlm.nih.gov]
  • Viral lymphomagenesis: from pathophysiology to the rationale for novel therapies. Br J Haematol. 2014;165(3):300–315. 62. Laurent C, Fabiani B, do C, et al.[dovepress.com]

Prevention

  • Awareness of this distinctive lymphoma can prevent misdiagnosis by the clinicians and/or the pathologists.[ncbi.nlm.nih.gov]
  • However, the histopathology and immunohistochemistry (IHC) of bone tissues from 2 separate biopsy sites revealed features of PBL. awareness to F-18 FDG PET/CT findings of PBL involving bones in an immunocompetent patient may prevent misdiagnosis.[ncbi.nlm.nih.gov]
  • […] cyclophosphamide 38 Ribavirin With or Without Monoclonal Antibody Therapy in Treating Patients Who Develop RSV Pneumonia Following Peripheral Stem Cell Transplantation Completed NCT00014391 Phase 3 ribavirin 39 Itraconazole Compared With Fluconazole to Prevent[malacards.org]
  • But there remain a number of unanswered questions and a need for improved prevention and therapy.[books.google.de]

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