Diffuse Large B-cell Lymphoma Arising in the Spleen

Diffuse Large B-cell Lymphoma Arising in the Spleen

Roberto N. Miranda, MD

Gross photograph shows a multinodular tumor image. Tumor extends into hilar fat image and shows focal infarction image. Well-preserved spleen is dark brown image. Spleen capsule is noted image.

Hematoxylin and eosin shows the most common presentation of DLBCL characterized by a well-circumscribed tumor mass image surrounded by nonneoplastic spleen image.



  • Diffuse large B-cell lymphoma (DLBCL)


  • Large cell lymphoma


  • Diffuse large B-cell lymphoma that arises in spleen

  • Involvement of splenic hilar lymph nodes and bone marrow (usually focal) can occur in primary DLBCL

    • Liver involvement may be part of definition of splenic DLBCL with micronodular or diffuse patterns

  • Primary DLBCL of spleen can be associated with splenic marginal zone B-cell lymphoma

  • Primary DLBCL of spleen is rare, but represents up to 40% of splenectomy specimens involved by DLBCL

  • Patients with history of lymphoma or evidence of disseminated DLBCL at diagnosis are excluded (secondary DLBCL)


Infectious Agents

  • No known etiology

  • Primary DLBCL of spleen is rarely associated with hepatitis C or Epstein-Barr virus infection



  • Age

    • Adults are mainly affected; median age: 64 years

      • M:F ratio approximately 1:1


  • Abdominal pain

  • Pain is often left-sided

  • Systemic symptoms such as fever, malaise, and weight loss often occur

  • Fine needle aspiration may yield necrosis only and be wrongly diagnosed as splenic abscess

  • Diagnosis can be suspected in presence of splenomegaly and abdominal or retroperitoneal adenopathy

  • Most patients are immunocompetent

    • Occasionally reported in HIV(+) patients


  • Chemotherapy similar to systemic cases of DLBCL

    • R-CHOP is most common chemotherapy used

  • Splenectomy usually performed for diagnostic purposes


  • 80% 5-year survival for patients with primary DLBCL presenting as mass

  • Poor survival for DLBCL with T-cell-rich pattern or neoplasms that diffusely replace spleen

    • These cases often have disseminated disease shown by staging (probably not primary)


General Features

  • Most cases show solitary or multiple distinct nodular masses surrounded by nonneoplastic spleen

    • Neoplasms that diffusely replace red pulp can be subtle grossly

  • Splenic weight can range from normal to > 3,000 g (average: 1,000 g)

  • Tumor size usually ranges from 5-18 cm

  • Multinodular tumor can replace up to 90% of spleen

  • Extensive necrosis is usual

  • Tumor may extend through capsule into adjacent diaphragm, stomach, pancreas, or abdominal wall


Histologic Features

  • Primary splenic DLBCL usually presents as large nodule or mass

    • Nodule/mass typically destroys white and red pulp

    • Approximately 1/3 of cases involve white pulp exclusively or predominantly

    • Approximately 20% of cases involve red pulp predominantly and diffusely

    • Adjacent Malpighian corpuscles may be focally involved

  • Variable cell morphology (centroblastic, immunoblastic, anaplastic, etc.)

    • Relative increased frequency of immunoblastic cases

  • Necrosis within neoplasm is common; sclerosis within or around neoplasm can be observed

  • Surrounding uninvolved spleen is distinctly separated from tumor, sometimes by fibrous bands

Predominant Pattern/Injury Type

  • Lymphoid, diffuse

Predominant Cell/Compartment Type

  • Hematopoietic, lymphoid


  • Neoplasms are of B-cell lineage: CD19(+), CD20(+), CD22(+), and pax-5(+)

  • Surface immunoglobulin light chain restriction is detected by flow cytometry in most cases

  • Most cases express Bcl-6 and about 1/3 express CD10

  • CD43 is positive in 20-30% of cases

  • CD3(−), CD5(−), CD23(−/+)

  • Absence of follicular dendritic cells (CD21, CD23) in tumor nodules

Cytogenetic and Molecular Findings

  • Monoclonal IgH rearrangements; TCR genes are usually germline

  • No distinctive cytogenetic or molecular findings


Diffuse Large B-cell Lymphoma (DLBCL), Systemic

  • Gross, microscopic, immunophenotypic, and molecular features can be identical to DLBCL arising in spleen

  • Distinction can be made after complete staging

  • Most DLBCL of spleen represent systemic or secondary involvement

T-cell/Histiocyte-rich Large B-cell Lymphoma (TCRLBCL)

  • Subtype of DLBCL that if identified in spleen, is suggestive of disseminated disease

  • Similar to any DLBCL both grossly and microscopically

  • Predominance of small T lymphocytes; large neoplastic B cells represent < 10% of cell infiltrate

  • Recently described micronodular variant (MTCRBL) does not produce large discrete mass but micronodules

  • MTCRBL distributed mainly in white pulp leaving no residual normal white pulp

DLBCL Primarily Involving Red Pulp

  • Unusual variant, clinically aggressive; median age: 69 years

  • Diffuse splenic involvement, predominantly in splenic cords

  • Frequent bone marrow and liver sinusoids infiltration; rare lymph node involvement

  • Mature B cells that usually coexpress CD5; Cyclin-D1 and CD23 negative

Peripheral T-cell Lymphoma (PTCL)

  • May involve white or red pulp

  • Cell composition is more polymorphic than DLBCL with mixture of eosinophils and plasma cells

    • Vascularity is often increased

  • Some cases may have increased histiocytes as well as erythrophagocytosis

  • Neoplastic cells including large cells react with T-cell markers (CD2, CD3, CD5, CD7); usually CD4(+)

Classical Hodgkin Lymphoma

  • Mainly involves white pulp

  • Scattered large Reed-Sternberg and Hodgkin cells in inflammatory background with eosinophils

  • Neoplastic cells are CD15(+), CD30(+), CD45(−), pax-5 (usually dim[+])

Nodular Lymphocyte-predominant Hodgkin Lymphoma (NLPHL)

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Diffuse Large B-cell Lymphoma Arising in the Spleen

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