Nodular Sclerosing Hodgkin Lymphoma and Mixed cellularity Hodgkin lymphoma
Oncology
InShort
Nodular Sclerosing Hodgkin Lymphoma
by Anurag Saraf, MD and Nancy Mills MD
AKA Nodular Sclerosis Classical Hodgkin Lymphoma
Nodular Sclerosing Hodgkin Lymphoma (NSHL) is the most common subtype of classical HL. Classical HL accounts for 95% of all HL (with Nodular Lymphocyte-Rich HL accounting for a majority of the remaining 5%). NSHL accounts for approximately 70% of all classical HL cases. NSHL presents commonly in younger patients (15-35 years old), occurs equally in males and females, and is commonly found in more affluent countries and patients with higher socioeconomic status.
Signs and Symptoms
NSHL most commonly presents with supradiaphragmatic lymphadenopathy, such as cervical and mediastinal lymph nodes, as well as lesions in the spleen, lung, liver, and bone marrow. Approximately 50% of patients present with bulky disease. Patients may present with what are called B symptoms (fevers, chills, night sweats) and diagnosis is commonly but not always at an early stage.
Diagnostic Evaluation
Diagnosis requires pathologic confirmation of disease. Microscopic hallmarks include collagen bands surrounding at least one nodule and Hodgkin Reed-Sternberg (HRS) cells with lacunar type morphology.
Prognosis
Prognosis of NSHL is generally good, with >90% survival at 5 years for early stage disease. Treatment often consists of chemotherapy, and advanced-disease is typically responsive to combination therapy with chemotherapy and radiation therapy. Patient who do not achieve complete remission with first-line therapy can typically be considered candidates for alternative chemotherapy regimens, targeted agents such as antibody-drug conjugate brentuximab, hematopoietic stem cell transplant or other cellular therapies, or immunotherapy such as PD-L1 inhibitors.
Further Reading
1. Luca, et al. “Nodular Sclerosis Classical Hodgkin Lymphoma”, Pathology Outlines.com. (http://www.pathologyoutlines.com/topic/lymphomanonBnshl.html).
2. Gansler, et al. “Hodgkin lymphoma: A review and update on recent progress” CA: A Cancer Journal for Clinicians. (https://onlinelibrary.wiley.com/journal/15424863).
Mixed cellularity Hodgkin lymphoma
by Anurag Saraf, MD, and Nirmala Saraf, MD
Mixed cellularity Hodgkin lymphoma (MCHL) is the second most common form of classical Hodgkin's lymphoma (HL). Classical HL accounts for 95% of all HL (with nodular lymphocyte-rich HL accounting for a majority of the remaining 5%). Further, nodular sclerosis HL accounts for most cases of classical HL, followed by MCHL, which accounts for ~20%-25% of classical HL cases. MCHL commonly presents in males, in patients affected with human immunodeficiency virus, and in individuals living in developing countries. The median age at diagnosis is 38 years.
Signs and Symptoms
MCHL commonly presents with peripheral lymphadenopathy, splenic and liver involvement, and supradiaphragmatic lymphadenopathy, especially cervical and mediastinal lymphadenopathy. Patients typically present with B symptoms (beta symptoms in those with lymphoma, lung cancer, and other cancers), such as fevers, chills, night sweats and unexplained weight loss.
Diagnostic Evaluation
Diagnosis of MCHL requires pathologic examination of tissue specimens. Reed-Sternberg tumor cells are typically seen in a background of a variety of inflammatory cells, including eosinophils, neutrophils, histiocytes, and plasma cells.
Reed–Sternberg cells (lacunar histiocytes) are characteristic giant cells usually derived from B lymphocytes. They are multinucleated and contain pronounced eosinophilic inclusion-like nucleoli. These large clonal tumor cells typically stand out in the midst of normal B lymphocytes.
Prognosis
Prognosis of patients with MCHL is intermediate when compared with those having other HL diseases, as patients respond to therapy. However, they typically present at an advanced stage with abdominal or splenic involvement. Treatment often consists of chemotherapy, and advanced disease typically responds to combination therapy with chemotherapy and radiation therapy.
More medical terminology on the FibonacciMD.app Compendium.
References
Carbone A, Gloghini A. Mixed cellularity classical Hodgkin lymphoma (MCcHL). Atlas Genet Cytogenet Oncol Haematol. 2017;21:98-102.
Luca DC. Nodular sclerosis classical Hodgkin lymphoma. Pathology Outlines.com Web site. http://www.pathologyoutlines.com/topic/lymphomanonBnshl.html. Accessed June 4, 2019.