Rare Diseases Symptoms Automatic Extraction

Diverse clinicopathologic features in human herpesvirus 8-associated lymphomas lead to diagnostic problems.

[primary effusion lymphoma]

Human herpesvirus 8 (HHV8)-associated lymphomas are uncommon, mainly affect men infected with the human immunodeficiency virus (HIV), and usually have a poor prognosis. We sought to characterize the HHV8+ lymphomas seen at our institution since the mid-1990s.We identified 15 patients with HHV8-associated lymphomas and evaluated their clinical and pathologic features.Diagnoses included primary effusion lymphoma (PEL) (n = 2), extracavitary PEL (n = 8), intravascular large B-cell lymphoma (n = 1), HHV8+ plasmablastic microlymphoma (n = 3), and germinotropic lymphoproliferative disorder (GLD) (n = 1). The case of GLD progressed to a high-grade HHV8+ Epstein-Barr virus-positive lymphoma, an evolution that has not been previously reported. Four patients were HIV-(three from an HHV8-endemic area). Potentially misleading pathologic features in our series of extracavitary PEL included classic Hodgkin lymphoma-like features, lymph node sinus involvement, and T-cell antigen expression.HHV8-associated lymphomas can be clinically and pathologically heterogeneous, with features that may lead to misdiagnosis as other types of lymphoma.

Diseases presenting "poor prognosis" symptom

  • adrenal incidentaloma
  • alexander disease
  • alpha-thalassemia
  • carcinoma of the gallbladder
  • cholangiocarcinoma
  • cushing syndrome
  • cutaneous mastocytosis
  • dedifferentiated liposarcoma
  • erdheim-chester disease
  • esophageal adenocarcinoma
  • esophageal carcinoma
  • esophageal squamous cell carcinoma
  • hodgkin lymphoma, classical
  • junctional epidermolysis bullosa
  • kindler syndrome
  • liposarcoma
  • locked-in syndrome
  • lymphangioleiomyomatosis
  • malignant atrophic papulosis
  • megacystis-microcolon-intestinal hypoperistalsis syndrome
  • papillon-lefèvre syndrome
  • pleomorphic liposarcoma
  • primary effusion lymphoma
  • pyomyositis
  • sneddon syndrome
  • systemic capillary leak syndrome
  • von hippel-lindau disease
  • waldenström macroglobulinemia
  • well-differentiated liposarcoma
  • werner syndrome
  • wiskott-aldrich syndrome
  • zellweger syndrome

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