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Table of Contents
Definition / general | Terminology | Epidemiology | Etiology | Clinical features | Laboratory | Radiology description | Radiology images | Prognostic factors | Case reports | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Peripheral smear description | Positive stains | Negative stains | Flow cytometry description | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosisCite this page: Hodgkin lymphoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/bonehod.html. Accessed July 14th, 2017.
Definition / general
- Hodgkin lymphoma (HL) is primarily a nodal disease - secondary involvement of bone can occur, is considered as Stage IV
- Primary bone HL is extremely rare (Ann Oncol 2008;19 Suppl 4:iv77)
Terminology
- Defined according to WHO criteria for primary bone lymphoma (PBL), as lymphoma presenting in an osseous site with no evidence of disease elsewhere for at least 6 months
- Involvement of regional lymph nodes does not exclude diagnosis of PBL (Joint Bone Spine 2000;67:446)
Epidemiology
- Bimodal age distribution, peaks in young adults and later stages of life
- Male to female ratio is slightly > 1
Etiology
- Associated with EBV (40% - 50% of cases, specific role not established)
- Also linked to HLA, associated with HIV infection, autoimmune disease, immunocompromise
Clinical features
- Bone pain, pathologic fracture, mass
- Neurologic symptoms in vertebral involvement
- Systemic symptoms ("B" symptoms) of fever, night sweats, weight loss
Laboratory
- Elevated ESR, elevated LDH
- Variable anemia
Radiology description
- Bone lesions are variable and non specific; may be lytic destructive, blastic sclerotic, or near normal patterns (Radiographics 2003;23:1371)
- Vertebral lesions spread from bone to bone, with intervertebral disc destruction
- CT and other radiologic studies useful for staging
Radiology images
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Osteoblastic involvement
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Osteolytic lesion
Increased uptake in the sternum
Sternal mass
Destruction of L1 vertebra
Loss of height of L1
Prognostic factors
- Stage of disease
- Systemic symptoms
- Tumor burden (bulky disease if > 10 cm)
- International Prognostic Score (IPS)
Case reports
- 7 year old boy with primary multifocal osseous lymphoma (World J Surg Oncol 2008;6:34)
- 21 year old man with primary multifocal osseous Hodgkin lymphoma (J Cancer Res Clin Oncol 2005;131:163)
- 35 year old man with iliac and sacral bone osteolytic lesions without other symptoms (J Cancer Res Ther 2013;9:511)
- 38 year old woman with primary HL in rib (Diagn Pathol 2012;7:64)
- 51 year old woman with left ileal mass (Am J Hematol 2006;81:550)
Treatment
- Combination of chemotherapy and radiotherapy
Microscopic (histologic) description
- Characteristic combination of Hodgkin Reed-Sternberg (HRS) cells (diagnostic cells), in background of mixed inflammatory cells
- HRS cells are large, binucleate or multinucleate cells, with thick nuclear membrane, gigantic eosinophilic inclusion surrounded by halo
- Variants:
- Mononuclear
- Lacunar (lobulated nuclei and retracted cytoplasm in formalin-fixed tissue)
- Pleomorphic (large bizarre nuclei)
- Mummified cells (discernible nuclear features and eosinophilic cytoplasm)
- Infiltrate of lymphocytes, plasma cells, histiocytes, eosinophils, and neutrophils
- Most common patterns are mixed cellularity (classic mononuclear HRS cells), and nodular sclerosing pattern (lacunar cells)
- May show accompanying fibrosis, granuloma
Microscopic (histologic) images
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Scattered lacunar cells
Mixed inflammatory cells
CD30+
Cytology description
- Spectrum of HRS cells in reactive inflammatory background
- Granulomas may be seen
Peripheral smear description
- Rouleaux formation, due to elevated ESR
- Neoplastic cells of HL rarely circulate in peripheral blood
Flow cytometry description
- Flow cytometry has no diagnostic value for HL itself, but may assist with differential diagnosis if cytology is not conclusive
- Recently, HRS-T cell rosettes identified by flow cytometry (Am J Clin Pathol 2006;126:764)
Electron microscopy description
- HRS cell cytoplasm contains numerous polyribosomes with coarsely condensed heterochromatin at nuclear membrane (J Cancer Res Clin Oncol 1981;101:135)
Molecular / cytogenetics description
- Gain of chromosome 2p, 12q, 17p, 9p, and 16p
Differential diagnosis
- Reactive conditions
- Anaplastic large cell lymphoma
- Diffuse large B cell lymphoma
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