Other lymphomas included in this category are:

• KSHV/HHV8-positive diffuse large B-cell lymphoma
• KSHV/HHV8-positive germinotropic lymphoproliferative disorder

Primary Effusion lymphoma

Introduction:

• It is a rare and aggressive B cell lymphoma usually affecting immunosuppressed individuals.

Epidemiology:

• Male predominance
• Usually seen in 20 to 50 years of age
• 4% of HIV related lymphomas

Etiology:

• Human Herpesvirus-8 / Kaposi’s sarcoma herpesvirus
• Many are seen in HIV patients
  
   

Clinical Features:

• Shortness of breath
• Findings consistent with pleural effusion
• Pericardial and peritoneal involvement is seen in some cases
• B-Symptoms
  
   

Investigations:

• Centrifuge preparations of pleural fluid
  • Tumor cells with immunoblastic cells to anaplastic morphology
  • Nuclei are large, round to irregular, with prominent nucleoli. Cytoplasm is very abundant & deeply basophilic. Vacuoles are seen in occasional cells. Sometimes perinuclearhof is seen.
• Immunophenotyping
  • Positive – Leukocyte common antigen (CD45), HHV8, EBER. Most express CD30, CD38/138, EMA, HLA-DR and EBER
  • Negative – Pan B Markers, T cell markers

Prognosis:

• Extremely unfavorable
• Median survival- <6months

Treatment:

• Chemotherapy: DA-EPOCH or CHOP
• ART for HIV positive patients
• Talc pleurodosis

KSHV/HHV8-positive diffuse large B-cell lymphoma

• Usually males; Age 30-40 years
• Often seen in HIV+ patients
• Micro: Large cells with plasmablastic or immunoblastic appearance
• IHC: CD20 +/-; MUM1+; IgM lambda+; CD138 negative; many vIL6 positive, EBER-Negative
• Prognosis: Very poor, with median survival of few months

KSHV/HHV8-positive germinotropic lymphoproliferative disorder

• Male:Female 1.5:1; Age 30-90 years
• Most are HIV negative
• Microscopy: 
  • Large cells with plasmablastic, immunoblastic, or anaplastic morphology
  • Variable replacement of germinal centers without significant nodal architectural effacement; may have scattered cells in interfollicular areas or sinuses
• IHC
  • Positive: MUM, vIL6, EBER
  • Negative: B-cell antigen, CD138, CD30
• No treatment is necessary. Without treatment also mean survival is around 15 years