Lymphomas: Pathology review

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An 8-year-old boy is brought to the clinic for evaluation of jaw swelling and an open wound on the cheek. The patient and family recently immigrated from Africa. The jaw wound has enlarged over the course of a few months and has been associated with intermittent fevers, fatigue, and weight loss. There was no recent trauma or insect bite to the area. Photograph of the wound is shown below. Biopsy of the jaw mass is obtained, and histopathology shows undifferentiated squamous epithelial cells forming a “starry sky” appearance. Which of the following best describes the underlying process causing this patient’s condition?
 
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At the family medicine center, there is a 25 year old male, named Hogan, who came in because of a painless non-erythematous mass on his neck. Next to Hogan, there is a 30 year old male immigrant from Africa, named Burak, who has noticed a painless mass on his jaw. He also complains of drenching night sweats, and unexplained weight loss over the last few months. Biopsy is ordered for both people. In Hogan’s there’s binucleated B cells surrounded by mainly lymphocytes. Burak’s biopsy showed numerous lymphocytes with some tingible-body macrophages. CBC is normal for both.

Both Hogan and Burak have lymphoma. Lymphomas are tumors derived from lymphocytes, which are B and T cells. They can be broadly grouped into two categories; Hodgkin and non-Hodgkin lymphomas.

In contrast, non-Hodgkin lymphomas don’t have Reed-Sternberg cells can sometimes spread non-contiguously, and can involve extranodal sites like the skin, gastrointestinal tract, and brain. Non-Hodgkin’s lymphomas can occur in both children and adults. Finally, overall prognosis is better with Hodgkin lymphomas.

Let’s start by looking at Hodgkin lymphoma. This type of lymphoma typically arise from B-cells and spread in a contiguous manner, meaning it spreads to nearby lymph nodes, and rarely involve extranodal sites. It has a bimodal age distribution, affecting young adults in their 20s and adults older than 60 years of age. Histologically, it’s characterized by the presence of Reed-Sternberg cells and for your exams, remember that these are binucleated, neoplastic B cells that look kind of like owl eyes. The large mononuclear version of Reed-Sternberg cells are called Hodgkin cells. These abnormal, neoplastic cells are usually surrounded by non-neoplastic inflammatory cells, mostly T cells, and sometimes eosinophils. They can also activate fibroblasts, which secrete collagen.

Sources

  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Fundamentals of Pathology" H.A. Sattar (2017)
  4. "Hodgkin lymphoma: A review and update on recent progress" CA: A Cancer Journal for Clinicians (2017)
  5. "Diffuse large B‐cell lymphoma: 2019 update on diagnosis, risk stratification, and treatment" American Journal of Hematology (2019)
  6. "The non-Hodgkin lymphomas: A review of the epidemiologic literature" International Journal of Cancer (2007)
  7. "The 2016 revision of the World Health Organization classification of lymphoid neoplasms" Blood (2016)
  8. "Review of the treatment of mycosis fungoides and Sézary syndrome: A stage-based approach. Int J Health Sci (Qassim)" Al hothali GI (2013)
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