Lymphoma and plasma cell neoplasms
Posttransplant lymphoproliferative disorders (PTLD)
Classic Hodgkin lymphoma PTLD

Editor-in-Chief: Debra Zynger, M.D.
Cade Arries, M.D.
Elizabeth Courville, M.D.

Topic Completed: 25 July 2019

Revised: 25 July 2019

Copyright: 2019, PathologyOutlines.com, Inc.

PubMed Search: Classical Hodgkin lymphoma PTLD

Cade Arries, M.D.
Elizabeth Courville, M.D.
Page views in 2019 to date: 214
Cite this page: Arries C, Courville E. Classic Hodgkin lymphoma PTLD. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomaclassichodgkinptld.html. Accessed October 17th, 2019.
Definition / general
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
Epidemiology
  • Very rare; the least common major form of PTLD, accounting for < 5% of all PTLD (Cancer Treat Res 2015;165:305, Leuk Lymphoma 2017;58:633)
  • Develops at any age
  • Occurs in both solid organ and allogeneic hematopoietic stem cell transplant recipients
  • Kidney and liver transplants are the most commonly reported in the literature
Sites
Pathophysiology
  • PTLDs in general develop as a consequence of immune suppression and decreased T cell immune surveillance
  • Classic Hodgkin lymphoma PTLD is almost always an EBV driven proliferation with only rare EBV- reported (Surg Case Rep 2018;4:72)
  • Has been reported to arise following diagnosis of polymorphic PTLD (Pediatr Transplant 2013;17:E168)
  • Exact pathophysiology of classic Hodgkin lymphoma PTLD is not entirely understood
Clinical features
  • Can include lymphadenopathy, splenomegaly, fever, weight loss, night sweats or pancytopenia
Diagnosis
  • Diagnosis is made most often on lymph node biopsy, less commonly with biopsy of extranodal tisssue and rarely on bone marrow biopsy
Prognostic factors
  • There are insufficient 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, nuclear contours are irregular (occasionally bilobed) or mononuclear and there are frequently prominent nucleoli
Microscopic (histologic) images

Contributed by Cade Arries, M.D.

Bone marrow

Bone marrow, CD30

Bone marrow, CD15

Lymph node


Lymph node

Lymph node, CD15

Lymph node, CD30

Lymph node, PAX5

Positive stains
  • In the Hodgkin Reed-Sternberg cells: PAX5 (dim), CD30 and CD15
  • B cell markers BOB.1, OCT2, CD79a are more commonly expressed in the large neoplastic cells of classic Hodgkin lymphoma PTLD versus classic Hodgkin lymphoma in immunocompetent hosts (100%, 86% and 50% compared with 6%, 14% and 10%, respectively) (Expert Opin Ther Targets 2009;13:1137)
Flow cytometry description
  • Hodgkin Reed-Sternberg cells are not identified by routine flow cytometric immunophenotyping
  • CD4:CD8 ratio of the background T cells may be increased (> 10:1)
Molecular / cytogenetics description
  • Recurrent cytogenetic / oncogene abnormalities are as yet unknown
  • In situ hybridization for Epstein-Barr virus (EBV) encoded small RNA (EBER1) is almost always positive
Molecular / cytogenetics images

Contributed by Cade Arries, M.D.

Bone marrow, EBER

Lymph node, EBER

Sample pathology report
  • Lymph node, cervical, excisional biopsy:
    • Classic Hodgkin lymphoma posttransplant lymphoproliferative disorder (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 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. Which of the following is the most appropriate diagnosis?



  1. Classic Hodgkin lymphoma posttransplant lymphoproliferative disorder
  2. Monomorphic posttransplant lymphoproliferative disorder (diffuse large B cell lymphoma)
  3. Nondestructive posttransplant lymphoproliferative disorder (florid follicular hyperplasia)
  4. Polymorphic posttransplant lymphoproliferative disorder
Board review answer #1
A. Classic Hodgkin lymphoma posttransplant lymphoproliferative disorder. The morphologic and immunohistochemical features are diagnostic of classic Hodgkin lymphoma. In the context of solid organ transplantation, this is best classified according to the revised 4th edition of the World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues as classic Hodgkin lymphoma posttransplant lymphoproliferative disorder. This is a rare disorder almost always showing EBV positivity by EBER in situ hybridization in the Reed-Sternberg cells / variants.

Reference: Classic Hodgkin lymphoma PTLD

Comment Here
Board review 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?

  1. ALK1
  2. CD3
  3. CD45
  4. EBER in situ hybridization
Board review 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.

Reference: Classic Hodgkin lymphoma PTLD

Comment Here
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