Lymphoma & related disorders

Posttransplant lymphoproliferative disorders (PTLD)

PTLD-classic Hodgkin


Editorial Board Member: Roberto N. Miranda, M.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Cade Arries, M.D.
Elizabeth Courville, M.D.

Last author update: 20 October 2022
Last staff update: 20 October 2022

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PubMed Search: Classical Hodgkin lymphoma PTLD

Cade Arries, M.D.
Elizabeth Courville, M.D.
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Cite this page: Arries C, Courville E. PTLD-classic Hodgkin. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomaclassichodgkinptld.html. Accessed February 4th, 2023.
Definition / general
  • Lymphoproliferation that fulfills the diagnostic criteria for classic Hodgkin lymphoma and arises in a patient after solid organ or hematopoietic stem cell transplant
  • Classification is unaltered in the 2022 International Consensus Classification of Mature Lymphoid Neoplasms (Blood 2022;140:1229)
  • In the 5th edition of the World Health Organization Classification of Hematolymphoid Tumors (WHO HAEM), the entity is classified based on a 3 part nomenclature for lymphomas that arise in the setting of immune deficiency / dysregulation (Leukemia 2022;36:1720)
Essential features
  • Fulfills the diagnostic criteria for classic Hodgkin lymphoma
  • Arises in a patient with a history of solid organ or allogeneic hematopoietic stem cell transplant
  • Almost always Epstein-Barr virus (EBV)+
  • Very rare; < 5% of all posttransplant lymphoproliferative disorders (PTLD)
ICD coding
  • ICD-O: 9650/3 - Hodgkin lymphoma, NOS
Epidemiology
Sites
Pathophysiology
Etiology
Clinical features
  • Can include lymphadenopathy, splenomegaly, fever, weight loss, night sweats or pancytopenia
  • Patients have a history of solid organ or hematopoietic cell transplant
Diagnosis
  • Diagnosis is made on lymph node biopsy (most common), with biopsy of extranodal tissue (less common) and on bone marrow biopsy (rarely)
Prognostic factors
  • There are an insufficient number of cases to draw meaningful conclusions
Case reports
Treatment
Microscopic (histologic) description
  • Resembles mixed cellularity subtype of classic Hodgkin lymphoma
  • Effaced architecture or interfollicular pattern
  • Variable interstitial fibrosis; less commonly with broad bands of sclerosis or capsular thickening
  • Typical Hodgkin Reed-Sternberg cells are seen scattered in a mixed inflammatory background of eosinophils, histiocytes, lymphocytes and plasma cells
    • Hodgkin Reed-Sternberg cells are large cells with irregular nuclear contours (occasionally bilobed) and frequently with prominent nucleoli
Microscopic (histologic) images

Contributed by Cade Arries, M.D.

Bone marrow

Bone marrow, CD30

Bone marrow, CD15

Bone marrow, EBER


Lymph node

Lymph node


Lymph node, CD15

Lymph node, CD30

Lymph node, PAX5

Lymph node, EBER

Positive stains
Flow cytometry description
Molecular / cytogenetics description
  • Recurrent cytogenetic / oncogene abnormalities are unknown at this time
Sample pathology report
  • Lymph node, cervical, excisional biopsy:
    • Classic Hodgkin lymphoma posttransplant lymphoproliferative disorder, EBV positive (see comment)
    • Comment: The morphologic and immunohistochemical findings fulfill the criteria for classic Hodgkin lymphoma. In this patient with a history of solid organ transplant, the findings are compatible with classic Hodgkin lymphoma posttransplant lymphoproliferative disorder. The Reed-Sternberg cells / variants are EBV+.
Differential diagnosis
Board review style question #1

A 45 year old man with a history of renal transplant presented with lymphadenopathy. A lymph node biopsy was performed. A representative image is shown. The large atypical cells are positive for CD30 and CD15 with dim PAX5 nuclear positivity and dim and variable CD20 expression. CD3 and CD45 are negative. What process is suggested by these morphologic and immunohistochemical findings?

  1. Classic Hodgkin lymphoma
  2. Diffuse large B cell lymphoma
  3. Florid follicular hyperplasia
  4. Polymorphic proliferation
Board review style answer #1
A. Classic Hodgkin lymphoma. The morphologic and immunohistochemical features are diagnostic of classic Hodgkin lymphoma. This is a rare disorder in the posttransplant setting and almost always shows EBV positivity by EBER in situ hybridization in the Reed-Sternberg cells / variants. In the context of solid organ transplantation, the posttransplant status and the presence or absence of viral association is noted in the interpretation. The exact nomenclature depends on the classification system used.

Comment Here

Reference: PTLD-classic Hodgkin
Board review style question #2
Which of the following is expected to be positive in the Hodgkin / Reed-Sternberg cells of classic Hodgkin lymphoma type posttransplant lymphoproliferative disorder (PTLD)?

  1. ALK1
  2. CD3
  3. CD45
  4. EBER in situ hybridization
Board review style answer #2
D. EBER in situ hybridization. The Hodgkin / Reed-Sternberg cells of classic Hodgkin lymphoma type posttransplant lymphoproliferative disorder are almost always EBV+ by EBER in situ hybridization. The other stains listed are negative.

Comment Here

Reference: PTLD-classic Hodgkin
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