Hodgkin Disease – Top USMLE Diseases

Lymphadenopathy is lymph node enlargement (> 1 cm) and is benign and self-limited in most patients. Etiologies include malignancy, infection, and autoimmune disorders, as well as iatrogenic causes such as the use of certain medications. Generalized lymphadenopathy often indicates underlying systemic disease. Diagnosis relies on a thorough medical history and physical examination, which usually identify the underlying cause. When the cause is unknown, a biopsy may be necessary. Management is directed at the underlying infection, malignancy, or other cause.

The myeloid lineage produces a diverse group of blood cells (leukocytes) involved in the defense of the body against foreign substances or infectious agents. Monocytes, neutrophils, eosinophils, basophils, and mast cells all arise from the common myeloid progenitor (CMP), which develops from bone marrow stem cells. However, leukocytes have different functions. Neutrophils kill invading bacteria and fungi, eosinophils increase in parasitic infections, basophils and mast cells are key to allergic response as sources of histamine, and monocytes are released from bone marrow to become macrophages in the tissues. The development in the bone marrow is regulated by different cytokines.

Lymphocytes are heterogeneous WBCs involved in immune response. Lymphocytes develop from the bone marrow, starting from hematopoietic stem cells (HSCs) and progressing to common lymphoid progenitors (CLPs). B and T lymphocytes and natural killer (NK) cells arise from the lineage. B and T lymphocytes play a role in adaptive immunity, and NK cells provide host defense against atypical proteins such as tumor cells. While all developmental stages begin in bone marrow, lymphocyte maturation differs. B lymphocytes and NK cells differentiate in bone marrow before migrating to secondary lymphoid organs (such as lymph nodes). T lymphocytes, however, proceed to the thymus for further maturation.

Hodgkin lymphoma (HL) is a malignancy of B lymphocytes originating in the lymph nodes. The pathognomonic histologic finding of HL is a Hodgkin/Reed-Sternberg (HRS) cell (giant multinucleated B cells with eosinophilic inclusions). The disease presents most commonly with lymphadenopathy (neck most commonly involved), night sweats, weight loss, fever, and at times, splenomegaly and hepatomegaly. Diagnostic testing includes lymph node histologic analysis showing HRS cells, blood tests, and CT and PET scanning. HL is managed with chemotherapy and radiotherapy. The prognosis has improved significantly with the advent of chemotherapeutic regimens.