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Hematolymphoid System

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Pathology of Childhood and Adolescence

Abstract

Hematopathology is the study of diseases and disorders affecting blood cells, their production, and any organs and tissues involved in hematopoiesis early in the embryonal age, such as the bone marrow, the spleen, and the thymus. In particular, text and illustrative material include more common and less common non-Hodgkin lymphomas, including Burkitt lymphoma, diffuse large B-cell lymphoma, and lymphoblastic lymphoma among others.

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Author information

Authors and Affiliations

  1. University of Alberta, Edmonton, AB, Canada

    Consolato M. Sergi

Authors
  1. Consolato M. Sergi
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Correspondence to Consolato M. Sergi .

Multiple Choice Questions and Answers

Multiple Choice Questions and Answers

  • HEM-1 A 12-year-old boy presents at the emergency department with pallor, low-grade fever, and enlargement of the spleen. The parents indicate that the child suffers from hereditary spherocytosis. His blood work shows a hemoglobin of 1.86 mmol/L (normal, 7.45–11.17 mmol/L or 12–18 g/dL), reticulocyte count of 2%, white blood cells at 6.0 x 109 cells per liter (6,000/mm3; normal range, 4.3–10.8 x 109 cells per liter), and platelet count of 200 x 109 cells per liter (200,000/ mm3; normal range, 150–400 x 109 cells per liter or 150,000–400,000/mm3). What is the most likely diagnosis?

    1. (a)

      Acute leukemia

    2. (b)

      Sepsis

    3. (c)

      Aplastic crisis

    4. (d)

      Acute splenic sequestration

    5. (e)

      Hemolytic crisis

  • HEM-2 Fanconi anemia (FA) is a rare inherited recessive disease, which is determined by mutations in one of fifteen genes. These genes are known to encode FA pathway components. In response to DNA damage, nuclear FA proteins associate into complexes of high molecular weight through a cascade of posttranslational modifications and molecular interactions. This results in the repair of damaged DNA. Which of the following features is NOT characteristic of FA?

    1. (a)

      Chromosome fragility

    2. (b)

      Infantile hematologic abnormalities

    3. (c)

      Pancytopenia

    4. (d)

      Skeletal anomalies

    5. (e)

      Squamous cell carcinoma of the head and neck

  • HEM-3 Which of the following disorders is associated with a factor extrinsic to the red blood cell?

    1. (a)

      Sickle cell anemia

    2. (b)

      Autoimmune hemolytic anemia

    3. (c)

      Glucose-6-phosphate dehydrogenase (G6PD) deficiency

    4. (d)

      Hereditary spherocytosis

    5. (e)

      Hereditary elliptocytosis

  • HEM-4 Which of the following statement on hereditary spherocytosis does NOT match with the disease?

    1. (a)

      Mitochondrial DNA pattern of inheritance.

    2. (b)

      The defect compromises the scaffolding of the red blood cell leading to a loss of membrane fragments with the formation of microspherocytes.

    3. (c)

      The microsporocyte membrane is hugely permeable to sodium.

    4. (d)

      Cholelithiasis and cholecystitis may develop in teenagers.

    5. (e)

      Splenectomy is the definitive therapy.

  • HEM-5 A 12-year-old boy presents with an enlargement of a right-sided lymph node persisting for more than 12 months. The child did not complain about any pain. The lymphadenopathy did not rise in the setting of an infection. There was no history of clinical symptoms, such as fever, malaise, night sweats, or weight loss. On physical examination, the lymph node was mostly soft and mobile. The lymph node was about 5 cm × 3 cm in size without apparent overlying skin changes. Laboratory testing showed a normal white blood cell count with elevated lymphocytes and positivity for Epstein-Barr virus (early immunoglobulin G), while Bartonella and Cytomegalovirus testing were negative. A biopsy was performed, and the histology of the lymph node is shown here:

figure a

  • What is the most likely diagnosis?

    1. (a)

      Castleman disease

    2. (b)

      Viral lymphadenitis

    3. (c)

      Langerhans cell histiocytosis

    4. (d)

      Hodgkin lymphoma

    5. (e)

      Diffuse large B-cell lymphoma

  • HEM-6 A 15-year-old boy develops an enlargement of a cervical lymph node for more than 6 months. Neither weight loss nor other B symptoms are noted. Microbiologically, all tuberculosis and sarcoidosis tests were negative. A biopsy was performed, and the histology of the lymph node is shown here:

figure b
figure c

  • What is the most likely diagnosis?

    1. (a)

      Hodgkin lymphoma, nodular sclerosis variant

    2. (b)

      Hodgkin lymphoma, depleted lymphocyte variant

    3. (c)

      Diffuse large B-cell lymphoma

    4. (d)

      Mantle cell lymphoma

    5. (e)

      Langerhans cell histiocytosis

  • HEM-7 What is the best combination of immunohistochemical markers to diagnose classical Hodgkin cells?

    1. (a)

      AE1–AE3, CD15, CD30, PAX5

    2. (b)

      CD15, CD30, PAX5, MUM-1

    3. (c)

      CD20, CD21, CD15, CD30

    4. (d)

      BOB-1, PAX5, MUM-1

    5. (e)

      CD15, CD30, CD56, CD138

  • HEM-8 Which of the following statements about mantle cell lymphoma (MCL) is TRUE?

    1. (a)

      Cyclin D1 is essential for the diagnosis of MCLs.

    2. (b)

      SOX11 is a useful marker in the diagnosis of MCL.

    3. (c)

      The proliferative index recognized by the mouse monoclonal antibody against Ki-67 (MIB-1 immunostaining) has no prognostic relevance.

    4. (d)

      Splenic involvement is rarely encountered as a presentation of patients harboring MCL.

    5. (e)

      B symptoms are common at the presentation of MCL

  • HEM-9 A 25-year-old woman who is an intravenous drug user brings to the emergency department her afebrile infant showing severe oral thrush. Upon examination, the infant’s blood work shows hypocalcemia on two repeated measurements, but white cell count seems to be within normal limits. What is the most likely diagnosis in this infant of the following ones proposed below?

    1. (a)

      Child abuse by the mother’s partner

    2. (b)

      Severe combined immunodeficiency disease with mutations in the tyrosine phosphatase CD45 gene

    3. (c)

      Chronic granulomatous disease

    4. (d)

      DiGeorge syndrome

  • HEM-10 Which of the following are the two best antibodies to apply in detecting bone marrow metastases and minimal residual disease of neuroblastoma?

    1. (a)

      PHOX2B and NB84

    2. (b)

      CD56 and CD57

    3. (c)

      NB84 and S100

    4. (d)

      NSE and S100

    5. (e)

      S100 and CD56

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Sergi, C.M. (2020). Hematolymphoid System. In: Pathology of Childhood and Adolescence. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-59169-7_10

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  • DOI: https://doi.org/10.1007/978-3-662-59169-7_10

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