Lymphoma

Definition

• Malignant disorder of lymphoid cells that reside predominantly in lymphoid tissues

• Hodgkin's lymphoma (HL) is distinguished from non-Hodgkin's lymphoma (NHL) by the presence of Reed-Sternberg (RS) cells

Clinical manifestations

• Lymphadenopathy (nontender)

HL: superficial (usually cervical/supraclavicular) - mediastinal lymphadenopathy:

nodal disease with orderly, anatomic spread to adjacent nodes NHL: diffuse; nodal and extranodal disease with noncontiguous spread; symptoms reflect involved sites (abdominal fullness, bone pain)

• Constitutional ("B") symptoms: fever ( -381). sweats, weight loss (>10% over 6 mos)

HL: periodic, recurrent "Pel-Ebstein" fever; 10-15% have pruritus NHL"B" symptoms less common than in HL

Diagnostic and staging evaluation

• Physical exam: lymph nodes, liver spleen size.Waldeyer's ring, testes (1% of NHL), skin

• Pathology: excisional lymph node bx (not FNA. need surrounding architecture) with immunophenotyping and cytogenetics; BM bx (except in HL clinical stage IA IIA with favorable features); LP if CNS involvement is clinically suspected

• Laboratory tests: CBC. BUN Cr. LFTs. ESR, LDH. uric acid. Ca. albumin

Consider HIV. HBV. HCV. HTLV. & EBV serologies and connective tissue diseases autoAbs

• Imaging: chest/abd/pelvic CT (but don't reliably detect spleen liver involvement)

need 2nd modality: gallium or PET scans head CT MRI if neurological symptoms; bone scan if bony pain or if A«i. elevated

Ann Arbor Staging System with Cotswolds Modifications

Stage

Features

1

Single lymph node (LN) region

II

-2 LN regions on the same side of the diaphragm

III

LN regions on both sides of the diaphragm

IV

Disseminated involvement of one or more extralymphatic organs

Modifiers: A = no symptoms; B fever, night sweats or weight loss; X bulky disease greatest transverse diam. of mediastinal mass max diam. of chest wall >1 3 on CXR or >10 cm if in abd; E = Involves single contiguous extranodal site; H = hepatic; S = splenic

Hodgkin's Lymphoma (HL) Epidemiology and risk factors

• 7.500 cases y. bimodal distribution (15-35 & >50 y); T male; ? role for EBV Pathology

• Affected nodes show RS cells (<1%) in background of non-neoplastic inflammatory cells

• Classic RS cells: bilobed nucleus & prominent nucleoli with surrounding dear space ("owl's eyes"). RS cells are clonal B-cells: CD 15 CD30 ♦. CD20- by flow cytometry.

Rye Histologic Classification of Classical HL

Lymphocyte predominance

5%

Abundant normal-appearing lymphocytes; mediastinal LAN uncommon; male predominance; good prognosis

Nodular sclerosis

60-80%

Collagen bands; frequent mediastinal LAN; young adults; female predominance: usually stage I II at dx

Mixed cellularity

15-30%

Pleomorphic; older age; male predominance; ^50% stage III VI at presentation; intermediate prognosis

Lymphocyte depletion

• 1%

Diffuse fibrosis and large numbers of RS cells; older, male patients: disseminated at dx; seen in HIV; worst prognosis

Nonclassical (5%): nodular lymphocyte predominant (NLP); involves peripheral LN 80% present in stage l-ll and Rx can be RT alone or ABVD • RT w 80% 10-y progression-free survival. 93% overall survival (/CO 1997;1S;3060) Consider rituximab as most NLP RS cells are CD20 ♦ Stage lll-IV treated as classical HL (see below)

Modalities

Combined Modality Therapy (CMT)

Chemo Alone Radiation Alone

Classical HL Stage l-ll Disease Treatment

Description

Usually ABVD • 4-6 cycles ± involved field radiotherapy (IFRT)

Stanford V + IFRT is an option as well

XRT should be strongly considered for all bulky sites

Emerging modality given long-term toxicities of XRT

2 cycles past best response (usually ABVD x 6)

Slightly worse disease-free survival vs. CMT. but overall survival same

Historically came first; now rarely used given long-term toxicities

Consider only for isolated stage IA disease_

(JCO 2005:23:6400)

HL Stage lll-IV International Prognostic Scorc (IPS) and Treatment

Prognosis IPS 5-y PFS

Chemotherapy regimens

4 51% 25 42%

ABVD (preferred 1st line) inejm iW2J27 h7i)

Consider BEACOPP if IPS -4 lijoorf 2004:i0m1»>

Consider to initial bulky sites or if PET © after chemotherapy

IPS (negative prognostic indicators): Alb ■ 4 g dl; Hb 10.5 g dl; male; age 45 y; Stage IV;WBC -15k (d; Lymph 600 (il or 8% of differential (n£#m !w8j»:1s06)

ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine): usually 6-8 cycles Stanford V (doxorubicin, bleomycin, vinblastine, vincristine, mechlorethamine. etoposide. prednisone): usually 3 cycles

BEACOPP (bleomycin, etoposide. doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone) in regular and escalated doses

Relapsed disease: salvage chemo. high-dose chemo • auto HSCT. alio HSCT Pts at risk for second malignancies: acute leukemia MDS. NHL, lung cancer (related to both radiation and chemotherapy), breast cancer (radiation)

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