Page 349 - Concise Pathology for Exam Preparation ( PDFDrive )

334 SECTION II Diseases of Organ Systems

TABLE 12.18. Classification of Hodgkin lymphoma—cont’d

Subtypes Morphology Immunophenotype Clinical features
LD Reticular variant: Many ‘diagnos- RS cells are CD15 and • Affects older males
tic RS cells’ and ‘variants’ with 30-positive; EBV- • Frequent association with HIV
paucity of background reactive positive infection
cells. • Usually present with advanced
Diffuse fibrosis variant: Hypocel- disease.
lular fibrillar background with • Constitute , 5% cases of HL
scattered ‘diagnostic RS cells’
and ‘variants’ and few reactive
cells
LP Frequent ‘L and H (popcorn) cells’ RS cells are CD 20-pos- • Young males
in the background of follicular itive, CD15-negative, • Cervical and axillary lymph-
dendritic cells and reactive B C D 3 0 - n e g a t i v e , adenopathy
cells EBV–BCL–6-positive • Rarely involve mediastinal
May transform into a large B cell (BCL6 is a germinal lymph nodes
lymphoma. centre specific tran-
scription factor)

Clinical Features
• Painless enlargement of lymph nodes, which are discrete, nontender and rubbery
• ‘Constitutional symptoms’ (fever, night sweats, unexplained weight loss of greater
than 10% body weight) are observed more with disseminated disease (Stages III and IV)
and mixed cellularity or lymphocyte depletion subtypes.
• Classical ‘Pel–Ebstein’ fever (fever showing cyclical pattern; several days or weeks of
fever alternating with afebrile periods) is rare. An uncommon paraneoplastic symptom
involves occurrence of pain in affected lymph nodes on consumption of alcohol.
• Cutaneous anergy due to depressed cell-mediated immunity may be seen.
Clinical Staging (Ann Arbor); (Table 12.19)

TABLE 12.19. Clinical Staging (Ann Arbor) of Hodgkin lymphoma

Stage Distribution of disease
(I) Involvement of single lymph node region (I) or involvement of a single extralymphatic organ or site (IE)
(II) Involvement of two or more lymph node regions on the same side of diaphragm, alone (II) or with
involvement of limited contiguous extralymphatic organs or tissue (IIE)
(III) Involvement of lymph node regions on both sides of diaphragm, which may include spleen (IIIS)
and/or limited contiguous extralymphatic organ/site (IIIE and IIIES)
(IV) Multiple or disseminated foci of involvement of one or more extralymphatic organs or tissues with
or without lymphatic involvement

Note: All stages are further divided on the basis of presence or absence of systemic symptoms into (A) and (B).
(Source: Robbins and Cotran Pathologic Basis of Disease, South Asia Edition, Vol II, Kumar, Abbas, Aster, Table 13-9, Page 611,
Copyright 2015.)

Prognosis and Treatment
• Tumour stage rather than type more important prognostic factor
• With current treatment protocols, cure rates of Stages I and II A—90%
• Advanced stage associated with a 60–70% disease-free survival for 5 years
• Complications of long-term chemotherapy and radiotherapy:
• Neoplastic complications: Increased rates of myelodysplastic syndrome, acute
leukaemia, lung cancer, NHL, breast and gastric carcinoma, sarcomas and malig-
nant melanoma
• Nonneoplastic complications: Pulmonary fibrosis and accelerated atherosclerosis
Clinical differences between HL and NHL are given in Table 12.20.

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