Hodgkin lymphoma: 2014 update on diagnosis, risk-stratification, and management.

[hodgkin lymphoma, classical]

Hodgkin lymphoma (HL ) is an uncommon B-cell lymphoid malignancy affecting 9 ,200 new patients annually and representing approximately 11 .5 % of all lymphomas in the United States .HL is composed of two distinct disease entities ; the more commonly diagnosed classical HL and the rare nodular lymphocyte-predominant HL . Nodular sclerosis , mixed cellularity , lymphocyte depletion , and lymphocyte-rich HL are subgroups under the designation of classical HL .An accurate assessment of the stage of disease in patients with HL is critical for the selection of the appropriate therapy . Prognostic models that identify patients at low or high risk for recurrence , as well as the response to therapy as determined by positron emission tomography scan , are used to optimize therapy .Initial therapy for HL patients is based on the histology of the disease , the anatomical stage , and the presence of poor prognostic features . Patients with early stage disease are treated with combined modality strategies using abbreviated courses of combination chemotherapy followed by involved-field radiation therapy , while those with advanced stage disease receive a longer course of chemotherapy often without radiation therapy . Management of relapsed /refractory disease : High -dose chemotherapy (HDCT ) followed by an autologous stem cell transplant (ASCT ) is the standard of care for most patients who relapse following initial therapy . For patients who fail HDCT with ASCT , brentuximab vedotin , palliative chemotherapy , nonmyeloablative allogeneic transplant , or participation in a clinical trial should be considered .

Diseases
Validation

Diseases presenting "high risk" symptom

22q11.2 deletion syndrome

acute rheumatic fever

adrenal incidentaloma

alpha-thalassemia

aniridia

canavan disease

congenital diaphragmatic hernia

congenital toxoplasmosis

cowden syndrome

cushing syndrome

cutaneous mastocytosis

esophageal adenocarcinoma

esophageal carcinoma

esophageal squamous cell carcinoma

fabry disease

harlequin ichthyosis

heparin-induced thrombocytopenia

hirschsprung disease

hodgkin lymphoma, classical

homocystinuria without methylmalonic aciduria

hydrocephalus with stenosis of the aqueduct of sylvius

krabbe disease

legionellosis

liposarcoma

locked-in syndrome

oligodontia

oral submucous fibrosis

papillon-lefèvre syndrome

pendred syndrome

phenylketonuria

primary hyperoxaluria type 1

severe combined immunodeficiency

sneddon syndrome

waldenström macroglobulinemia

werner syndrome

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