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PBL 6 Quiz. 1a. A 9-year-old boy with fatigue, epistaxis, and easy bruisability develops pneumonia. On examination, he also has extremity bone pain, mild splenomegaly, and generalized nontender lymphadenopathy. His peripheral blood smear is shown.
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1a. A 9-year-old boy with fatigue, epistaxis, and easy bruisability develops pneumonia. On examination, he also has extremity bone pain, mild splenomegaly, and generalized nontender lymphadenopathy. His peripheral blood smear is shown. What condition is he likely to have and what enzyme is expressed in these cells? Terminal deoxynucleotidyltransferase (TdT) is expressed only in pre-B and pre-T lymphoblasts.
Answer: These cells (arrow) have condensed chromatin, minimal agranular cytoplasm with enlarged nuclei having small nucleoli with acute lymphoblastic leukemia. Early pre-B cell ALL (80% of cases)
b. Explain his clinical findings; i.e. fatigue, epistaxis, easy bruisability, extremity bone pain, mild splenomegaly, and generalized nontenderlymphadenopathy
Leukemic cells fill the marrow and displace the normal hematopoietic elements, leading to pancytopenia; the result is anemia with fatigue, thrombocytopenia with bleeding, and decreased myelopoiesis leading to bacterial infections. c. Are B or T cells most commonly implicated in ALL?
2. A 51-year-old woman has diffuse nontender lymphadenopathy. The microscopic appearance of a lymph node biopsy specimen is shown.
This is a follicular lymphoma, the most common form of non-Hodgkin lymphoma (about 45% of cases). Note the nodular expansion of the relatively monomorphous tumor cells. Note the nodular aggregates that are present throughout the lymph node. Unlike germinal centers, these nodules are composed of a monomorphic infiltrate of lymphoma cells
b. Transformation to what disease may occur in this patient? c. Is this an example of Richter syndrome?
Thirty percent to 50% transform to diffuse large B-cell lymphoma. No, Richter syndrome is when 5-10% of Chronic Lymphocytic Leukemia transform in diffuse large B-cell lymphoma.
3. A 6-year-old girl has a 1-month history of fatigue, headache, and vomiting. Her hemoglobin is 8g/dL (nl 12 to 15.6g/dL), platelet count is 56,400/mL (nl 130,000 to 400,000/mL), and total WBC count is 18,270/mL (nl 3800 to 10,800/mL). Flow cytometry of a marrow aspirate exhibits the pattern shown.
What condition is she likely to have? • What is the prognosis with chemotherapy? What would be some negative prognostic factor?
The red population of cells marks for CD19 (a B-cell marker) and CD10 (a pre-B cell marker) consistent with acute pre-B cell lymphoblastic leukemia. There is a smaller green population of normal B cells with remaining normal marrow cells highlighted here in gray. • Her prognosis is good. Worse prognostic factors include age younger than 2 years or onset in adolescence or adulthood, higher WBC count, and presence of the t(9;22)(Philadelphia chromosome) translocation.
A 44-year-old man has painless lumps in his upper posterior neck for the past year. His head CT scan is shown.
4a. What does this CT scan demonstrate? Posterior cervical lymphadenopathy b. What is the diagnosis if a biopsy specimen shows Reed-Sternberg cells? Hodgkin lymphoma, most common site of initial involvement is the neck region
Reed-Sternberg cells • Neoplastic cell of HL • Transformed germinal centre B cell (some cases) • CD15 and CD 30 positive • Classic RS cell; two mirror image neuclei, each with an eosinophilic nucleolus surrounded by a clear halo
5. A 61-year-old woman with fever, rigors, and tachycardia has a blood culture positive for Enterobacter cloacae. Her peripheral blood smear is shown. Inflammatory cytokines such as tumor necrosis factor and interleukin-1 stimulate bone marrow stromal cells to produce growth factors, such as granulocyte-macrophage colony-stimulating factor (GM-CSF), which drive myeloid proliferation.
6. A 59-year-old man with a 3-month history of dysphagia has a head CT scan showing enlargement of lymphoid tissues in Waldeyer?s ring. A biopsy specimen (shown) yields cells that immunostain for CD20 and BCL6. He is treated with intensive chemotherapy and remains in remission after 5 years. What are Waldeyer’s ring? Which type of Non-Hodgkin lymphoma commonly affects this site?
Diffuse Large B-Cell Lymphoma can arise anywhere in the body but commonly present in Waldeyer ring, the oropharyngeal lymphoid tissue that includes the tonsils and adenoids.
What structures are enlarged in this abdominal CT (arrow is a hint of where to look)? Given that this patient is a 24-year-old man with a 4-day history of abdominal pain, fever, diarrhea, and anorexia has the abdominal CT scan shown. His stool cultures are positive for Yersinia enterocoliticawould you say this is pathological? The enlarged mesenteric lymph nodes represent a reactive lymphadenitis.
