Lymphoma & related disorders

Hodgkin lymphoma

CHL lymphocyte depleted



Last author update: 25 July 2022
Last staff update: 25 July 2022

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PubMed Search: Lymphocyte depleted classic Hodgkin lymphoma

Anna B. Owczarczyk, M.D., Ph.D.
Lauren Smith, M.D.
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Cite this page: Owczarczyk AB, Smith L. CHL lymphocyte depleted. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomanonBlymphocytedepleted.html. Accessed December 9th, 2022.
Definition / general
  • Subtype of classic Hodgkin lymphoma (CHL) with numerous Reed-Sternberg cells and scant nonneoplastic background lymphocytes
Essential features
Terminology
  • Hodgkin lymphoma disease, lymphocyte depletion, NOS
  • Classic Hodgkin lymphoma, lymphocyte depletion, NOS
  • Hodgkin lymphoma, lymphocyte depletion, diffuse fibrosis
  • Hodgkin lymphoma, lymphocyte depletion, reticular
ICD coding
  • ICD-O:
    • 9653/3 - Hodgkin lymphoma, lymphocyte depletion, NOS
    • 9654/3 - Hodgkin lymphoma, lymphocyte depletion, diffuse fibrosis
    • 9655/3 - Hodgkin lymphoma, lymphocyte depletion, reticular
  • ICD-10: C81.30 - lymphocyte depleted Hodgkin lymphoma, unspecified site
  • ICD-11: 2B30.13 - lymphocyte depleted classical Hodgkin lymphoma
Epidemiology
Sites
Pathophysiology
Clinical features
Diagnosis
  • Excisional tissue biopsy or resection
  • Fine needle aspiration or core biopsy may be difficult to distinguish between CHL subtypes given the significance of tissue architecture and background inflammatory infiltrate for diagnosis
Laboratory
Radiology description
Radiology images

Images hosted on other servers:
Mesenteric and retroperitoneal lymphadenopathy

Mesenteric and retroperitoneal lymphadenopathy

Prognostic factors
  • Unfavorable prognostic factors (J Clin Oncol 2011;29:3914):
    • Advanced disease stage
    • Advanced age
    • Presence of B symptoms
    • ≥ 3 lymph node areas involved
    • Large mediastinal mass
    • High ESR (erythrocyte sedimentation rate)
    • HIV positive
Case reports
Treatment
Microscopic (histologic) description
  • Predominance of Reed-Sternberg cells and scarce background lymphocytes
    • Reed-Sternberg cells have multilobated nuclei with prominent eosinophilic inclusion-like nucleoli
  • May have coagulative necrosis or sinusoidal invasion
  • 2 patterns:
    • Diffuse fibrosis:
      • Prominent fibroblastic proliferation (nonbirefringent fibrillary stroma) without well formed fibrous bands
      • Numerous histiocytes
      • Scattered Reed-Sternberg cells
      • Scant lymphocytes
      • Lack of plasma cells or eosinophils
    • Reticular:
      • Rich in Reed-Sternberg cells (often sheets) with anaplastic, pleomorphic or sarcomatous features
      • Scant background small lymphocytes
      • Capsular and perinodal infiltration is common
  • References: Leuk Lymphoma 2009;50:937, Pathol Res Pract 2013;209:201
Microscopic (histologic) images

AFIP images
Diffuse fibrosis

Diffuse fibrosis

Numerous Hodgkin cells

Numerous Hodgkin cells

Cytology description
  • Predominantly large, atypical cells with irregular / convoluted nuclear contour, binucleation / multinucleation and frequent mitotic figures
  • Rare small lymphocytes
Positive stains
Negative stains
Flow cytometry description
  • Usually noncontributory as large Hodgkin and Reed-Sternberg cells are delicate and frequently destroyed during flow cytometric processing
Electron microscopy description
Electron microscopy images
Molecular / cytogenetics description
  • High rate of IGH gene rearrangements, likely related to the high density of Reed-Sternberg cells relative to nonneoplastic lymphoid cells in the specimen (Leuk Lymphoma 2009;50:937)
Sample pathology report
  • Retroperitoneal lymph node, excisional biopsy:
    • Classic Hodgkin lymphoma, lymphocyte depleted type (see comment)
    • Comment: H&E sections show an effaced lymph node by sheets of large, bizarre cells with irregular, multilobated nuclei, vesicular chromatin, prominent nucleoli and abundant cytoplasm. Rare background small lymphocytes are noted.
    • Immunohistochemical stains show that the atypical cells are positive for CD30, CD15 (subset), PAX5 (weak) and EBER (in situ hybridization for EBV encoded RNA). The atypical cells are negative for CD20, CD45, OCT2, BOB1 and ALK1. CD3 highlights rare small T cells.
    • Concurrent flow cytometric analysis was negative for monoclonal B cells or aberrant T cells.
Differential diagnosis
Board review style question #1
A middle aged HIV+ man presents with abdominal pain and a large retroperitoneal mass is detected on imaging. Core biopsy was performed, showing sheets of bizarre, atypical cells with large nucleoli and frequent binucleation. Scattered background small lymphocytes are present. The atypical cells are positive for CD30, EBER and PAX5 (weak) but negative for CD20. Molecular studies demonstrate immunoglobulin gene rearrangement. What is the most likely diagnosis?

  1. Anaplastic large cell lymphoma
  2. Classic Hodgkin lymphoma, lymphocyte depleted
  3. Classic Hodgkin lymphoma, lymphocyte rich
  4. Diffuse large B cell lymphoma, NOS
  5. EBV positive diffuse large cell lymphoma
Board review style answer #1
B. Classic Hodgkin lymphoma, lymphocyte depleted

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Reference: CHL lymphocyte depleted
Board review style question #2

A 35 year old man has extensive subdiaphragmatic lymphadenopathy. Core biopsy of one of the lymph nodes shows an effaced architecture with diffuse, disordered fibrosis and abundant histocytes. Scattered large, binucleated, CD30 positive and EBER positive atypical cells are noted. Plasma cells, eosinophils and small lymphocytes are essentially absent. Which of the following is true about this disease entity?

  1. Atypical cells are strongly positive for PU.1, BOB1, CD79a and CD20
  2. Best detected by flow cytometry
  3. More common in developed nations
  4. Most likely occurs in female patients
  5. Often associated with HIV infection
Board review style answer #2
E. Often associated with HIV infection

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Reference: CHL lymphocyte depleted
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