Hodgkin’s Lymphoma

Definition

Hodgkin lymphoma (HL), also referred to as Hodgkin disease, is an uncommon hematologic malignancy arising from mature B cells.

characterized by the presence of Hodgkin cells and Reed-Sternberg cells.WHO Classification:

Classical Hodgkin lymphoma (95% of cases).Within this category:

Nodular sclerosis (70%)

Mixed cellularity (25%)

Lymphocyte-rich (5%)

Lymphocyte-depleted (<1%).

Nodular lymphocyte-predominant Hodgkin lymphoma (5% of cases).

Etiology & Pathophysiology

Unclear

Likely Multi-factorialEBV

Environmental Factors

Epidemiology

HL accounts for approximately 10 percent of all lymphomas

approximately 0.6 percent of all cancers diagnosed in the developed world annually

incidence is higher in males than in females

difference in subtype based on age

Data from: National Cancer Institute. SEER Cancer Statistics Review 1975-2008. Surveillance Epidemiology and End Results. http://seer.cancer.gov/csr/1975_2008/index.html (Accessed February 16, 2012).

Clinical FeaturesAsymptomatic lymphadenopathy Mediastinal mass

chest pain, cough, shortness breath or combination may be present

Systemic symptoms“B” symptomspruritus and fatigue may be present

Intermittent fever is observed in approximately 35% of cases;

infrequently, the classic Pel-Ebstein fever is observed (high fever for 1-2 wk, followed by an afebrile period of 1-2 wk)

Pain at sites of nodal disease, precipitated by drinking alcohol, occurs in fewer than 10% of patientsBack or bone pain may rarely occurFamily history (NSHL genetic component)Hepatomegaly/Splenomegaly may be present

Workup

Laboratory TestsCBC (Anemia (normochromic / normocytic), eosinophilia, neutrophilia, lymphopenia

Elevated ESR

Metabolic Panel

Imaging StudiesCXR

CT Scan

PET Scan

LN FNAC / biopsy : Malignant REED-STERNBERG ( RS) Cell: Bi-nucleate cell with a prominent nucleolus. Derived from B cell, at an early stage of differentiation

Reactive background of eosinophils, lymphocytes, plasma cells

Fibrous tissue

Images

CXR of patient presenting with dyspnea, showing widened mediastinum and tracheal displacementCT level of neck showing Hodgin LymphomaCT/PET/MIP histologically proven Hodgin Lymphoma

Histological Findings

MHCL showing both mononucleate and binucleate Reed-Sternberg cells in a background of inflammatory cells

Staging

Cotswolds modification to Ann Arbor staging systemStaging with definition:

I: involvement of a single lymph node region (I) or lymphoid structure (IAE) (e.g., spleen, Waldeyer ring)II: involvement of 2 or more lymph node regions on the same side of the diaphragm (II)III: involvement of lymph node regions or structures on both sides of the diaphragm with (IIIE) or without involvement (III)IV: involvement of extranodal site(s) beyond that designated E.

Annotations:A: no B symptomsB: fever, drenching night sweats, weight lossX: bulky disease (>one third widening of mediastinum at T5 and T6, or >10-cm nodal mass)E: involvement of a single extranodal site, contiguous or proximal to known nodal site

Treatment & Prognosis

RT

Chemo

BMT / SCT

Antibody treatment: Rituximab target CD-20

Supportive

Overall 10 yr survival – 80%

In long term survivors there is a risk ofsecondary malignancy: (leukemia , NHL), Solid tumors- Lung, breastInfectionsCardiac, pulmonary, endocrinal abnormalities

References

NCCN Clinical Practice Guidelines in Oncology: Hodgkin lymphoma. Version 2.2014. National Comprehensive Cancer Network. Available at http://www.nccn.org/professionals/physician_gls/pdf/hodgkins.pdf.

First Aid for the Medicine Clerkships (2013)

https://online.epocrates.com/u/2963311/Hodgkin+lymphoma

Ayala, Carlos MD. Boards and Wards. 4th Edition: Wolters Kluwer.