The outlook, even for those with advanced disease, is very good. The 5-year survival rate for patients with stage I-A or II-A disease is more than 90%. Even with advanced disease (stage IV-A or IV-B), the overall 5-year disease-free survival rate is around 50%. Among long-term survivors treated with radiotherapy, a higher risk of certain malignancies, including lung cancer and breast cancer, as well as cardiovascular disease, has been reported. These sobering results have spurred development of new regimens that minimize the use of radiotherapy and use less toxic chemotherapy. As already mentioned, anti-PD-1 antibodies have produced excellent responses in patients with relapsed,
Table 12.10 Clinical Staging of Hodgkin and Non-Hodgkin Lymphomas (Ann Arbor Classification)
Stage Distribution of Disease
IIIInvolvementoflymphnoderegionsonbothsidesofthediaphragm(III),whichmayincludethespleen(IIIS),limitedcontiguousextralymphaticorganorsite(IIIE),orboth(IIIES) aAllstagesarefurtherdividedbasedontheabsence(A)orpresence(B)ofthefollowingsystemicsymptomsandsigns:significantfever,nightsweats,unexplainedlossofmorethan10%ofnormalbodyweight.FromCarbonePT,etal:Symposium(AnnArbor):staginginHodgkindisease.Cancer Res 31:1707,1971.
http://ebooksmedicine.net refractory disease and represent a promising immune-based therapy.
Among the many other forms of lymphoid neoplasia in the WHO classification, several with distinctive or clinically important features merit brief discussion.
Hairy Cell Leukemia. Hairy cell leukemia is an uncommon, indolent B cell neoplasm characterized by the presence of fine, hairlike cytoplasmic projections. The tumor cells express B cell markers (CD20), surface immunoglobulin, and CD11c and CD103; the latter two antigens are not present on most other B cell tumors, making them diagnostically useful. Virtually all cases are associated with activating mutations in the serine/threonine kinase BRAF, which is also mutated in diverse other cancers (Chapter 6).
Hairy cell leukemia occurs mainly in older males, and its manifestations result from infiltration of bone marrow and spleen. Splenomegaly, often massive, is the most common and sometimes only physical finding. Pancytopenia, resulting from marrow infiltration and splenic sequestration, is seen in more than half of cases. Lymph node involvement is rare. Leukocytosis is uncommon, being present only in 15% to 20% of patients, but scattered “hairy cells” can be identified in the peripheral blood smear in most cases. The disease is indolent but progressive if untreated; pancytopenia and infections are the major clinical problems. Unlike most other indolent lymphoid neoplasms, hairy cell leukemia is extremely sensitive to chemotherapeutic agents, particularly purine nucleosides. Complete durable responses are the rule and the overall prognosis is excellent. Tumors that fail conventional therapy have excellent responses to BRAF inhibitors, which ultimately may become the treatment of choice.