Paediatrics Marrow Pg 211-220 (Childhood Malignancies)
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Questions and Answers

What is a key factor in the development of diabetic ketoacidosis?

  • Severe insulin deficiency (correct)
  • Increased physical activity
  • High carbohydrate intake
  • Excessive hydration

    • Elevated levels of growth hormone contribute to insulin sensitivity in diabetic ketoacidosis.

    False

    Name one of the three ketone bodies formed during diabetic ketoacidosis.

    Acetone

    What appearance do café-au-lait spots have in neurofibromatosis-1?

    <p>Regular borders resembling the coastline of California</p> Signup and view all the answers

    In diabetic ketoacidosis, the condition known as ___________ is characterized by rapid and deep breathing.

    <p>Kussmaul's breathing</p> Signup and view all the answers

    Match the following symptoms and criteria with their corresponding descriptions:

    <p>Hyperglycemia = Blood glucose &gt; 200 mg/dL Acidosis = Venous pH &lt; 7.3 or serum bicarbonate &lt; 18 mmol/L Ketonemia = β-HB ≥ 5.3 mmol/L Fruity odor of breath = Distinctive breath odor associated with DKA</p> Signup and view all the answers

    Aromatase inhibitors are used to increase estrogen levels in McCune Albright syndrome.

    <p>False</p> Signup and view all the answers

    What is the primary medical management for central precocious puberty?

    <p>Long-acting GnRH analogue (e.g., Leuprolide)</p> Signup and view all the answers

    In the treatment of McCune Albright syndrome, the use of ______ inhibitors decreases estrogen synthesis.

    <p>aromatase</p> Signup and view all the answers

    Match the following conditions with their treatment:

    <p>McCune Albright syndrome = Aromatase inhibitors Central precocious puberty = Long-acting GnRH analogue Neurofibromatosis-1 = Medical management of underlying condition General hormonal disorders = Hormone replacement therapy</p> Signup and view all the answers

    Which type of leukemia is the most common in children?

    <p>Acute lymphoblastic leukemia</p> Signup and view all the answers

    Environmental factors like ionizing radiation are a risk factor for Acute lymphoblastic leukemia.

    <p>True</p> Signup and view all the answers

    What is the typical age group affected by Acute lymphoblastic leukemia?

    <p>2-5 years</p> Signup and view all the answers

    __________ syndrome is associated with an increased risk for Acute lymphoblastic leukemia.

    <p>Down's</p> Signup and view all the answers

    Match the cell types in Acute lymphoblastic leukemia with their corresponding CD markers:

    <p>Pre B cell = CD10, CD19, CD20, CD45 Mature B cell = CD19, CD20, CD21 T cell = CD2, CD3, CD4, CD5, CD7, CD8</p> Signup and view all the answers

    Which of the following chemotherapeutic agents is considered high-risk treatment for childhood malignancies?

    <p>Methotrexate</p> Signup and view all the answers

    Langerhans Cell Histiocytosis primarily affects the liver and kidneys.

    <p>False</p> Signup and view all the answers

    What is the common clinical feature seen in the pituitary gland due to Langerhans Cell Histiocytosis?

    <p>Diabetes Insipidus</p> Signup and view all the answers

    Langerhans Cell Histiocytosis is also known as _____ X.

    <p>Histiocytosis</p> Signup and view all the answers

    Match the following stages of lymph node involvement with their descriptions:

    <p>Stage 1 = No lymph node involvement Stage 2 = Single lymph node involvement Stage 3 = Multiple lymph nodes involvement Stage 4 = Multiple lymph nodes involvement</p> Signup and view all the answers

    What is the most common type of lymphoma worldwide?

    <p>Burkitt's lymphoma</p> Signup and view all the answers

    T cell type lymphomas are primarily associated with jaw lesions.

    <p>False</p> Signup and view all the answers

    Name one common clinical feature of Burkitt Lymphoma in its endemic form.

    <p>Jaw lesions</p> Signup and view all the answers

    Non-Hodgkin's lymphoma can be classified into B cell and T cell types. One of the T cell types is ___________.

    <p>Lymphoblastic lymphoma</p> Signup and view all the answers

    Match the following Non-Hodgkin's lymphoma types with their respective characteristics:

    <p>Burkitt's lymphoma = Endemic to Africa, jaw lesions Diffuse large B cell lymphoma = Most common form in adults Lymphoblastic lymphoma = Most common in India Anaplastic large cell lymphoma = Associated with T cell origin</p> Signup and view all the answers

    What is a common clinical feature of hematological malignancies similar to Acute Lymphoblastic Leukemia (ALL)?

    <p>Very high WBC count leading to leukemia-associated complications</p> Signup and view all the answers

    Disseminated Intravascular Coagulation (DIC) is commonly seen in m4 varieties of hematological malignancies.

    <p>False</p> Signup and view all the answers

    What is the primary drug used in the induction phase of treatment for hematological malignancies?

    <p>Cytosine arabinoside</p> Signup and view all the answers

    Hodgkin's Lymphoma has a male predominance and typically affects individuals in the age group of ______ to ______.

    <p>15 to 19 years</p> Signup and view all the answers

    Match the type of lymphoma with its description:

    <p>Hodgkin's Lymphoma = Presence of Reed-Sternberg cells Non-Hodgkin Lymphoma = Absence of Reed-Sternberg cells Chloroma = Deposition of leukemia cells in skin Acute Promyelocytic Leukemia (APML) = Use of ATRA in treatment</p> Signup and view all the answers

    Which of the following is a common clinical feature of childhood malignancies?

    <p>Painless lymphadenopathy</p> Signup and view all the answers

    Nodular sclerosis is one of the subtypes of Classical Hodgkin Lymphoma.

    <p>True</p> Signup and view all the answers

    What is the primary positive CD marker associated with malignant transformation in Hodgkin Lymphoma?

    <p>RS cell</p> Signup and view all the answers

    In the modified Ann Arbor staging, Stage II indicates _______ lymph nodes affected on one side of the diaphragm.

    <p>≥2</p> Signup and view all the answers

    Match the following subtypes of Classical Hodgkin Lymphoma with their characteristics:

    <p>Nodular sclerosis = Most common subtype Mixed cellularity = Balance of lymphocytes and other cell types Lymphocyte-rich = Contains many lymphocytes Lymphocyte-depleted = Poor prognosis</p> Signup and view all the answers

    What is the primary goal of the induction phase in the treatment of childhood malignancies?

    <p>To induce remission</p> Signup and view all the answers

    Cytosine-arabinoside is used in the maintenance phase of treatment for childhood malignancies.

    <p>False</p> Signup and view all the answers

    List two favorable risk factors for Acute Myeloid Leukemia.

    <p>Inversion 16, +(8; 21)</p> Signup and view all the answers

    The goal of the consolidation phase in treating childhood malignancies is to tackle __________.

    <p>drug resistance</p> Signup and view all the answers

    Match the following medications to their associated treatment phase in childhood malignancies:

    <p>Prednisolone = Induction phase Methotrexate = Prophylactic CNS therapy Vincristine = Induction phase Cyclophosphamide = Consolidation phase</p> Signup and view all the answers

    Which of the following is a known risk factor for Acute lymphoblastic leukemia?

    <p>Ionizing radiation</p> Signup and view all the answers

    Langerhans Cell Histiocytosis primarily affects the brain and heart.

    <p>False</p> Signup and view all the answers

    Which symptom is NOT typically associated with bone marrow failure?

    <p>Excess leukocytes</p> Signup and view all the answers

    What is a common clinical feature seen in the pituitary gland due to Langerhans Cell Histiocytosis?

    <p>Diabetes insipidus</p> Signup and view all the answers

    Hepatosplenomegaly indicates infiltration of the liver and spleen in hematological malignancies.

    <p>True</p> Signup and view all the answers

    The type of leukemia that is most common in children is ____________.

    <p>Acute lymphoblastic leukemia</p> Signup and view all the answers

    What is the minimum percentage of lymphoblasts required for the diagnosis of Acute Lymphoblastic Leukemia (ALL)?

    <p>25%</p> Signup and view all the answers

    Match the following conditions with their corresponding treatment:

    <p>McCune Albright Syndrome = Aromatase inhibitors Acute lymphoblastic leukemia = Chemotherapy Langerhans Cell Histiocytosis = Steroids Central precocious puberty = Gonadotropin-releasing hormone agonists</p> Signup and view all the answers

    The condition characterized by compression of the Superior Vena Cava leads to __________.

    <p>SVC syndrome</p> Signup and view all the answers

    Match the following features with their corresponding prognosis classification:

    <p>CNS involvement = High risk/poor prognosis Initial WBC count &lt; 50,000/mm³ = Standard risk/good prognosis Age &gt; 10 years = High risk/poor prognosis Gender: Female = Standard risk/good prognosis</p> Signup and view all the answers

    Which of the following features indicates a high risk/poor prognosis classification in hematological malignancies?

    <p>Male gender</p> Signup and view all the answers

    Presence of hepatosplenomegaly indicates that the bone marrow is failing.

    <p>False</p> Signup and view all the answers

    What type of swelling is associated with testicular leukemia?

    <p>Painless unilateral or bilateral testicular swelling</p> Signup and view all the answers

    Study Notes

    Pubertal Disorders

    • Treatment for pubertal disorders includes addressing the underlying condition and medical management.
    • McCune Albright syndrome can be treated with aromatase inhibitors, such as Letrozole, to inhibit estrogen synthesis.
    • Central precocious puberty can be treated with long-acting GnRH analogues, such as Leuprolide, which desensitize the pituitary and decrease LH and FSH production, ultimately reducing sex steroid levels.

    Diabetic Ketoacidosis (DKA)

    • DKA is characterized by severe insulin deficiency or absence, leading to hyperglycemia and ketoacidosis.
    • Glucose draws water from intracellular compartments to the extracellular space, leading to intracellular dehydration and pseudohyponatremia.
    • Precipitating factors for DKA include infection and trauma.
    • Counter-regulatory hormones, such as growth hormone, glucagon, and cortisol, contribute to insulin resistance and lipolysis.
    • Hyperglycemia stimulates lipolysis, leading to the production of fatty acids, which are oxidized in the liver to form ketone bodies.
    • Ketone bodies, including acetone, acetoacetate, and β-hydroxy butyrate, can cause abdominal pain and a fruity odor of breath.
    • Kussmaul's breathing, characterized by rapid and deep breathing, is a compensatory mechanism to reduce acidosis.

    DKA Diagnostic Criteria

    • Hyperglycemia: Blood glucose > 200 mg/dL
    • Acidosis: Venous pH < 7.3 or serum bicarbonate < 18 mmol/L
    • Ketones: Ketonemia (β-HB ≥ 5.3 mmol/L) or ketonuria (≥ 2+ on dipstick)

    Hematological Malignancies

    • Leukemia is the most common hematological malignancy, characterized by clonal proliferation and arrest of maturation of immature cell series.
    • Acute leukemia is the most common type, including acute lymphoblastic leukemia (ALL) and acute myeloblastic leukemia (AML).
    • Leukemia is the most frequent malignancy in children, particularly in the 2-5 year age group, with males being more affected than females.
    • Conditions associated with an increased risk of ALL include Ataxia telangiectasia, Bloom’s syndrome, severe combined immunodeficiency (SCID), Down’s syndrome, environmental factors such as ionizing radiation, Fanconi’s anemia, and Li Fraumeni syndrome.

    Classification of ALL

    • Immunophenotypic Classification: Based on cell type and identified via flow cytometry.
      • Pre B cell (most common, 85%): CD10 positive, CD19, CD20, and CD45.
      • Mature B cell: CD19, CD20, and CD21.
      • T cell: CD2, CD3, CD4, CD5, CD7, and CD8.
    • Cytogenetic classification: Based on chromosome number and translocation.

    St. Jude Staging for ALL

    • The St Jude staging system classifies ALL based on lymph node involvement and other lesions.
    • Lymph Node Involvement
      • Stage 1: Single lymph node affected.
      • Stage 2: Multiple lymph nodes affected on the same side of the diaphragm.
      • Stage 3: Multiple lymph nodes affected on both sides of the diaphragm.
      • Stage 4: Multiple lymph nodes with involvement of bone marrow and/or CNS.
    • Other Lesions
      • Stage 1: No other lesions.
      • Stage 2: Single, resectable gastrointestinal lesion.
      • Stage 3: Non-resectable gastrointestinal lesions.
      • Stage 4: Non-resectable gastrointestinal lesions with bone marrow and/or CNS involvement.

    Treatment for ALL

    • Malignant chemotherapy:
      • Cyclophosphamide: An alkylating agent that damages DNA.
      • Vincristine: A microtubule inhibitor that prevents cell division.
      • Prednisolone: A corticosteroid that suppresses the immune system and inflammation.
      • Doxorubicin: An anthracycline antibiotic that prevents DNA replication.
      • Methotrexate: A folate analog that interferes with DNA synthesis.

    Langerhans Cell Histiocytosis (LCH)

    • Also known as Histiocytosis X, LCH is a clonal proliferation of Langerhans-like cells.
    • LCH can be multisystemic and affect various sites:
      • Bone: Skull is the most affected, followed by the jaw, leading to missing or loose teeth.
      • Skin: Exhibit scales and discharge, sometimes mimicking seborrheic dermatitis and unresponsive to typical treatment.
      • Pituitary: Infiltration can lead to stalk destruction, blocking vasopressin/ADH release, resulting in diabetes insipidus (DI).

    AML (Acute Myeloid Leukemia)

    • Involves the clonal proliferation of immature myeloid cells.
    • Can present similarly to ALL, but hepatosplenomegaly is less common.
    • There is a higher risk for leukostasis, leading to lung infiltrates and stroke manifestations.
    • Disseminated Intravascular Coagulation (DIC) can occur in the m3 variety of AML.
    • Gum hypertrophy is observed in the m4 subtype.
    • Diagnosis is confirmed via bone marrow aspiration, with >20% myeloblasts indicating AML.

    Treatment for AML

    • Includes induction, consolidation, and maintenance phases.
    • Induction Phase: Aims to induce remission and observe response, with the goal of reducing Minimal Residual Disease (MRD):
      • Cytosine arabinoside: An antimetabolite that inhibits DNA synthesis.
      • Daunorubicin: An anthracycline that inhibits DNA replication.
    • Drugs used in m3 (APML):
      • ATRA (All-trans retinoic acid)/tretinoin: A retinoid that promotes differentiation of blast cells into mature cells.
      • Cytosine arabinoside: An antimetabolite that inhibits DNA synthesis.
      • Etoposide: A topoisomerase inhibitor that prevents DNA replication.
    • Consolidation Phase: Aims to eliminate drug resistance and maintain remission.
      • Methotrexate: A folate analog that disrupts DNA synthesis.
      • Epiodophyllotoxin: A topoisomerase inhibitor that prevents DNA replication.
      • Cyclophosphamide: An alkylating agent that damages DNA.
      • Cytosine-arabinoside: An antimetabolite that inhibits DNA synthesis.
      • 6-mercaptopurine: An antimetabolite that inhibits DNA synthesis.
      • Methotrexate: A folate analog that disrupts DNA synthesis.
    • Maintenance Phase: Aims to prevent relapse.
      • Oral 6-mercaptopurine and methotrexate: Once a week for a year.

    Lymphoma

    • Hodgkin's Lymphoma (HL): Characterized by the presence of Reed-Sternberg cells (RS cells) and affects children aged 15-19 years, with a male predominance.
    • Risk factors for HL:
      • Infections: HHV6, CMV, and EBV (increase risk by 4x).
      • Autoimmune disorders: Systemic lupus erythematosus (SLE).
    • Non-Hodgkin’s Lymphoma (NHL): Characterized by absence of Reed-Sternberg cells, and affects children aged 5-15 years.
      • Based on lymphocyte of origin:
        • B cell type: Includes Burkitt’s lymphoma (most common worldwide) and Diffuse large B cell lymphoma (DLBCL).
        • T cell type: Includes Lymphoblastic lymphoma (most common in India), Anaplastic large cell lymphoma, and Intermediate cell lymphoma.

    Burkitt's Lymphoma (BL)

    • Associated with Epstein-Barr virus (EBV).
    • Endemic BL: Present worldwide (endemic to Africa), with a variety of presentations:
      • Jaw lesions: Lytic lesions of bones, draining sinuses, and diffuse swelling.
      • Generalized abdominal distension due to intra-abdominal tumors.
    • Sporadic BL (In India):
      • Intra-abdominal tumors.
      • Mass in the pelvis and abdomen.
      • Unexplained abdominal pain.
      • Intussusception with tumors as the lead point.

    Clinical Features of HL

    • Painless, matted, and firm rubbery cervical lymphadenopathy.
    • Constitutional symptoms including high-grade fever (> 38°C), drenching night sweats, and weight loss (>10% in the last 6 months).
    • Positive CD Markers: RS cells on excisional biopsy of lymph node, indicating malignant transformation of a germinal B cell.

    Types of HL

    • Classical HL:
      • Nodular sclerosis HL: CD15 and CD30 positive, with owl eye inclusion bodies.
      • Nodular-lymphocyte predominant HL: CD20 and CD45 positive with popcorn cells.
    • Subtypes of classical HL:
      • Nodular sclerosis
      • Mixed cellularity
      • Lymphocyte-rich
      • Lymphocyte-depleted
    • RS cell variants:
      • Lacunar: Mononuclear and reticular cells. Excellent prognosis.
      • Good: -
      • Excellent: -
      • Poor: -

    Treatment for HL

    • Based on modified Ann Arbor staging.

    Modified Ann Arbor Staging for HL

    • Stage I: Single lymph node affected.
    • Stage II: ≥2 lymph nodes on one side of the diaphragm.
    • Stage III: Multiple lymph nodes above and/or below the diaphragm.
    • Stage IV: Multiple lymph nodes with organ involvement.

    Treatment for HL

    • Treatment varies based on stage and risk factors.
    • Favorable Risk: Includes chemotherapy with the "ABVD regimen" (Adriamycin, Bleomycin, Vinblastine, Dacarbazine).
    • Unfavorable Risk: Includes chemotherapy and radiotherapy.
    • "X" classification: Bulky tumor (size >10 cm).
    • "E" classification: Extension into extralymphatic sites.

    General Features of Leukemia

    • Bone marrow failure leading to:
      • Anemia
      • Thrombocytopenia (present as petechiae)
      • Leukopenia
      • Exception: Leukocytes may be seen in a few patients.
    • Night bone pains (due to leukemia cell infiltration).

    Extramedullary Involvement of Leukemia

    • Occurs in >60% of cases.
    • Liver and spleen infiltration: Hepatosplenomegaly.
    • Superficial lymphadenopathy: Non-tender, firm, fixed, and confluent.
    • Mediastinal involvement:
      • Tracheal compression: Unexplained cough and breathing difficulty.
      • SVC syndrome: Compression of the superior vena cava (SVC) leading to venous stasis, resulting in facial flushing and edema, and jugular vein distension.
    • CNS leukemia:
    • Testicular leukemia: Painless, unilateral, or bilateral testicular swelling.
    • Cranial neuropathies:
      • Seizures.
      • Increased intracranial pressure features.

    Diagnosis of Leukemia

    • Bone marrow aspiration and biopsy:
      • Aspiration may be normal.
      • ≥ 25% lymphoblasts: Diagnosis of ALL.

    Classification of ALL Based on Prognosis

    • Standard risk/good prognosis (≤10 years, Female, < 50,000/mm³):
      • Age: 2-10 years.
      • Gender: Female.
      • CNS involvement: No.
      • Initial WBC count: < 50,000/mm³.
    • High risk/poor prognosis (≤10 years, Male, > 50,000/mm³):
      • Age: ≤ 1 year and > 10 years.
      • Gender: Male.
      • CNS involvement: Yes.
      • Initial WBC count: > 50,000/mm³.

    Subtypes of ALL

    • Cytogenetics:
      • Pre-B cell:
        • Hyperdiploidy (> 50 chromosomes).
        • Trisomy 4 and 10.
      • B cell and T cell:
        • Hypoploidy (reduced number of chromosomes).

    Treatment for ALL

    • Divided into phases: induction, prophylactic CNS therapy, consolidation, and maintenance.
    • Induction Phase: Goal is to induce remission and observe response.
      • Prednisolone: A corticosteroid that suppresses the immune system and inflammation.
      • L-asparaginase: A bacterial enzyme that depletes asparagine, an amino acid crucial for leukemia cell growth.
      • Anthracycline: An anthracycline antibiotic that inhibits DNA replication.
      • Vincristine: A microtubule inhibitor that prevents cell division.
    • Prophylactic CNS therapy: Goal is to treat subclinical CNS involvement.
      • Intrathecal methotrexate: A folate analog administered directly into the cerebrospinal fluid.
    • Consolidation (Intensification) Phase: Aims to tackle drug resistance and sustain remission.
      • Methotrexate: A folate analog that disrupts DNA synthesis.
      • Epiodophyllotoxin: A topoisomerase inhibitor that prevents DNA replication.
      • Cyclophosphamide: An alkylating agent that damages DNA.
      • Cytosine-arabinoside: An antimetabolite that inhibits DNA synthesis.
      • 6-mercaptopurine: An antimetabolite that inhibits DNA synthesis.
      • Methotrexate: A folate analog that disrupts DNA synthesis.
    • Maintenance Phase: Goal is to prevent relapse.
      • Oral 6-mercaptopurine and methotrexate: Once a week for a year.

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    Description

    This quiz covers key concepts related to pubertal disorders, including treatment options for conditions like McCune Albright syndrome and central precocious puberty. Additionally, it explores the mechanisms and precipitating factors of Diabetic Ketoacidosis (DKA). Test your knowledge on both topics and understand their clinical implications.

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