Hodgkin Disease
Background
At what age does Hodgkin disease (HD) most commonly occur?
HD has a bimodal peak with peaks at age 25 yrs and age 60–70 yrs.
What are 2 broad histologic categories of HD? Which is more common?
Broad histologic categories of HD:
Classic (more common)
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL)
What are the subtypes of classic HD, and which is most common in the U.S.?
Subtypes of classic HD:
Nodular sclerosing (most common in the U.S.)
Mixed cellularity
Lymphocyte depleted
Lymphocyte rich
What are the 2 most commonly involved LN regions at the initial Dx of HD?
Most commonly involved LN regions at initial Dx of HD:
Cervical chains (70% of pts)
Mediastinum (50% of pts)
Pts who present with mediastinal LAD are most likely to have which subtype of HD?
Pts who present with mediastinal LAD are most likely to have nodular sclerosing HD.
In classic HD, what is the most common CD15, -30, -45, and -20 staining pattern?
In classic HD, tumors are typically CD15 and -30 positive but CD45 and -20 negative.
In NLPHL, what is the most common CD15, -30, -45, and -20 staining pattern?
In NLPHL, tumors are typically CD15 and -30 negative but CD45 and -20 positive (i.e., the reverse of classic HD).
Which HD subtype has the best prognosis?
Lymphocyte-rich HD has the best prognosis.
Which HD subtype has the worst prognosis?
Lymphocyte-depleted HD has the worst prognosis.
Which HD subtype is associated with older age or HIV+ pts?
Lymphocyte-depleted HD is associated with older age and HIV+ pts.
Pts with which subtype of HD are at greatest risk of developing a subsequent non-Hodgkin lymphoma?
Pts with NLPHL are at greatest risk of developing a subsequent non-Hodgkin lymphoma.
What are the “B Sx” of lymphoma?
B Sx include:
Fevers >38°C
>10% body weight loss in 6 mos
Drenching night sweats
How is bulky mediastinal Dz commonly defined?
Bulky mediastinal Dz is commonly defined as a mass greater than one third of the intrathoracic diameter at T5-6 on upright PA film.
How is bulky Dz defined outside of the mediastinum?
Outside of the mediastinum, bulky Dz is variably defined in clinical trials but most often is either any mass >5 cm or any mass >10 cm.
Workup/Staging
What kind of Bx is preferred for Dx of HD and why?
Excisional Bx is preferred for the Dx of lymphomas b/c it shows LN architecture.
What imaging studies are typically ordered as part of the workup of HD?
An integrated PET/CT is commonly used in the workup imaging for HD.
What lab work is required as part of the workup of HD?
The following labs have prognostic implications: ESR, CBC, albumin, and LDH.
Labs necessary for Tx planning are BUN, Cr, and a pregnancy test in women of childbearing age.
What are common indications for a BM Bx in the workup of HD?
Common indications for a staging BM Bx:
B Sx
Stages III–IV
Bulky Dz
>2 sites
Recurrent Dz
How is HD staged?
HD is staged using the Ann Arbor system:
Stage I: involvement of 1 LN region or localized involvement of a single extralymphatic organ or site (IE)
Stage II: involvement of ≥2 LN regions on same side of diaphragm or localized involvement of a single associated extralymphatic organ or site and its regional LN with or without involvement of other LN regions on same side of diaphragm (IIE)
Stage III: involvement of LN regions on both sides of diaphragm that may also be accompanied by localized involvement of an associated extralymphatic organ or site (IIIE)
Stage IV: multifocal involvement of ≥1 extralymphatic organ, with or without associated LN involvement, or isolated extralymphatic organ involvement with distant nodal involvement.
Note: Pts without B Sx are designated with an A, otherwise with a B. Pts with splenic involvement are designated with an S.
Involvement of what sites is considered stage 4 Dz?
Per the AJCC (7th edition), pts with involvement of the BM, liver, pleura, and CSF have stage IV Dz.
Name the 14 distinct LN regions as per the Rye classification.
LN regions per the Rye classification:
Waldeyer ring
Occipital, cervical, preauricular, and supraclavicular
Infraclavicular
Axillary
Epitrochlear
Mediastinum
Right hilum
Left hilum
Para-aortic
Spleen
Mesenteric
Iliac
Inguinofemoral
Popliteal
Is involvement of the Waldeyer ring and spleen considered extranodal?
No. Per the AJCC (7th edition), the Waldeyer ring and spleen are not classified as extranodal sites.
What does the Waldeyer ring include?
The Waldeyer ring includes:
Pharyngeal tonsil (adenoids)
Palatine tonsil
Lingual tonsil (base of tongue)