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Thus, the precise stage of such a patient would be CS IIB, PS IVB (S )(H )(M ).

The non-Hodgkin lymphomas (NHL) are a heterogeneous group of lymphoproliferative malignancies with differing patterns of behavior and responses to treatment.[1] Like Hodgkin lymphoma, NHL usually originates in lymphoid tissues and can spread to other organs.

Pruritus as a systemic symptom remains controversial and is not considered a B symptom in the Ann Arbor staging system.

(Refer to the PDQ summary on Pruritus for more information.) This symptom is hard to define quantitatively and uniformly, but when it is recurrent, generalized, and otherwise unexplained, and when it ebbs and flows parallel to disease activity, it may be the equivalent of a B symptom.] The designation E is used when well-localized extranodal lymphoid malignancies arise in or extend to tissues beyond, but near, the major lymphatic aggregates.

Clinical staging for patients with Hodgkin lymphoma (HL) includes a history, physical examination, laboratory studies (including sedimentation rate), and thoracic and abdominal/pelvic computerized tomographic (CT) scans with or without positron emission tomography (PET).[1,2] PET scans combined with CT scans have become the standard imaging for clinical staging.[2] A prospective, multinational study of 260 newly diagnosed patients with advanced-stage HL obtained PET scans at baseline and after two cycles (four doses) of doxorubicin plus bleomycin plus vinblastine plus dacarbazine (ABVD); with a median follow-up of 2.2 years, the 2-year progression-free survival (PFS) was 12.8% with a positive PET scan after two cycles and 95% with a negative PET scan after two cycles (10 years) to assess long-term toxicities or treatment-related mortality.[9] Bone marrow involvement occurs in 5% of patients; biopsy may be indicated in the presence of constitutional B symptoms or anemia, leukopenia, or thrombocytopenia.

In a retrospective review and meta-analysis of 955 patients in nine studies, fewer than 2% of patients with a positive bone marrow biopsy had only stage I or II disease on PET-CT scans; omission of the bone marrow biopsy for PET-CT–designated early-stage patients did not change treatment selection.[10] Staging laparotomy is no longer recommended and should be considered only when the results will allow substantial reduction in treatment.

The aggressive type of NHL has a shorter natural history, but a significant number of these patients can be cured with intensive combination chemotherapy regimens.Staging laparotomy should not be done in patients who require chemotherapy.If the laparotomy is required for treatment decisions, the risks of potential morbidity should be considered.[11-14] The staging classification that is currently used for HL was adopted in 1971 at the Ann Arbor Conference [15] with some modifications 18 years later from the Cotswolds meeting.[1] Subclassification of stage Stages I, II, III, and IV adult HL can be subclassified into A and B categories: B for those with defined general symptoms and A for those without B symptoms.More than 75% of all newly diagnosed patients with adult HL can be cured with combination chemotherapy and/or radiation therapy.[2] National mortality has fallen more rapidly for adult HL than for any other malignancy over the last 5 decades.[2] Prognosis for a given patient depends on several factors.The most important factors are the presence or absence of systemic symptoms, the stage of disease, presence of large masses, and the quality and suitability of the treatment administered.Limitation of radiation dose and fields and avoidance of leukemogenic chemotherapeutic agents, along with watchful waiting policies, should be investigated for these subgroups.[8,17,18] For patients with advanced-stage NLPHL, chemotherapy regimens designed for patients with non-HLs may be preferred, based on two retrospective reviews and a phase II study.[19-21][Level of evidence: 3iii Dii] Rituximab had a 100% response rate in a phase II trial of 39 previously untreated and relapsed NLPHL patients.With a median follow-up of 9.8 years, the median PFS was 3.0 years for patients who received rituximab induction only and 5.6 years for patients who received rituximab induction plus rituximab maintenance.[10][Level of evidence: 3iii Diii] With induction only, 9 of 23 patients relapsed with an aggressive B-cell lymphoma.Patients, however, can often be re-treated with considerable success as long as the disease histology remains low grade.Patients who present with or convert to aggressive forms of NHL may have sustained complete remissions with combination chemotherapy regimens or aggressive consolidation with marrow or stem cell support.[5,6] Late effects of treatment for non-Hodgkin lymphoma (NHL) have been observed.The vast majority of relapses occur in the first 2 years after therapy.The risk of late relapse is higher in patients who manifest both indolent and aggressive histologies.[4] While indolent NHL is responsive to immunotherapy, radiation therapy, and chemotherapy, a continuous rate of relapse is usually seen in advanced stages.

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