H o d g k i n D i s e a s e
General Characteristics
Terminology
• Hodgkin Disease is the same as Hodgkin Lymphoma. You can use the
terms interchangeably.
• Don't say "Hodgkin's Disease" though. Leave the apostrophe and s off the
end of disease names in general, unless the person who named the disease
actually had the disease. This is a fairly new rule in disease terminology, but
it's rapidly being adopted as the correct way to state things. (You'll note
Robbins names diseases in this fashion.)
Clinical Features
• Arises in a single lymph node/nodal chain and usually spreads to contiguous
lymph nodes. Eventual spread to spleen, liver, bone marrow, other organs
may occur.
• Rare overall (3/100,000), but one of most common malignancies in young
adults (“bimodal” distribution: 1 peak at age 20-30 years, another peak in
50s - except in Nodular Lymphocyte Predominance Hodgkin Lymphoma;
see below).
The Reed-Sternberg (R-S) cell
• The R-S cell is the malignant cell in Hodgkin's Disease (the background cells
are benign and probably reactive). Its exact origin is unknown, but people
pretty much agree that it is a lymphoid cell of some type.
• Necessary (need to see them) but not sufficient (other diseases can have
similar cells) for diagnosis.
• Very large cell; two or more nuclei with surrounding, clear "halos" and big
nucleoli; abundant, amphophilic cytoplasm.
Subtypes
• nodular lymphocyte predominance Hodgkin lymphoma
• classical Hodgkin lymphoma
Classical Hodgkin lymphoma is further subdived into four types:
• Nodular sclerosis Hodgkin lymphoma
• Lymphocyte-rich classical Hodgkin lymphoma
• Mixed cellularity Hodgkin lymphoma
• Lymphocyte depletion Hodgkin lymphoma
Nodular Lymphocyte Predominance Hodgkin Lymphoma (NLPHL)
This type of Hodgkin Lymphoma is separated out from the other, "classic" forms of Hodgkin Lymphoma for several reasons:
• Lymphocyte-predominant HL is usually diagnosed in asymptomatic young
(30's - 40's) males with enlarged cervical lymph nodes (in classic HL, there
is a bimodal age distribution, and disease is not limited to the cervical lymph
nodes).
• Patients with NLPHL generally have earlier-stage disease, longer survival,
and fewer treatment failures than those with classic HL.
• It is of B-cell origin. The typical immunophenotype for NLPHL is CD15-,
CD20+, CD30-, CD45+ (the profile for classic HL is CD15+, CD20-, CD30+,
CD45-).
Nodular Sclerosis Hodgkin Lymphoma
• Most common subtype (>60% of all cases of Hodgkin Lymphoma).
• Clinical Features: Many patients present with limited disease; prognosis is
excellent.
• Morphology:
• Lacunar cells (R-S variants with single, hyperlobated nucleus and
abundant, pale-staining cytoplasm. Formalin fixation makes the
cytoplasm retract, and the cell appears to be sitting in a little space, or
lacuna). Classic R-S cells are rare.
• Background: small lymphocytes and eosinophils.
• Collagen bands divide lymph node tissue into nodules (hence, "nodular
sclerosis").
Mixed Cellularity Hodgkin Lymphoma
• Second most common subtype (>20% of all cases of Hodgkin Lymphoma).
• Clinical Features: Many patients present with disseminated disease and
systemic symptoms.
• Morphology:
• Classic R-S cells are plentiful.
• Background: eosinophils, plasma cells, histiocytes.
• Sort of a “wastebasket” category: lots of different appearances.
Lymphocyte Rich Hodgkin's Lymphoma
• Third most common subtype (<10% of all cases of Hodgkin Lymphoma).
• Clinical Features: Most patients present with limited disease; prognosis is
excellent.
• Morphology:
• Characterized by popcorn cell (R-S variant with delicate, multilobated,
puffy nucleus).
• Classic R-S cells are hard to find (but present).
• Background: mature lymphocytes with variable numbers of histiocytes.
Eosinophils, neutrophils, plasma cells are scanty or absent.
Lymphocyte Depletion Hodgkin's Disease
• Least common subtype (<5% of all cases of Hodgkin's Lymphoma).
• Clinical features: Patients often older; many present with disseminated
involvement and systemic symptoms; prognosis is poor.
• Morphology:
• Classic R-S cells and R-S variants are numerous.
• Background contains either collagen or reticulin fibers; lymphocytes are
rare.
Therapy and Prognosis
Therapy
• Surgical removal of lymph node (if disease is limited)
• Chemotherapy
• Radiation therapy
Prognosis
• Depends on stage. If disease is localized, 5-year survival is about 90%.
If disease is disseminated, 5-year survival is about 60%.
• Morphologic subtype is not independently important in predicting prognosis!
(It just happens that some subtypes present with limited disease and some
present with widespread disease.)
• Relatively long survival means patients have time to develop second
malignancies (most commonly acute myeloid leukemia) related to
chemotherapy and radiation. These are very hard to cure.
