hodgkin lymphoma
TRANSCRIPT
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.