Medicine Marrow Pg 151-160 (Hematology)
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Medicine Marrow Pg 151-160 (Hematology)

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Which characteristic is specific for Hodgkin's Lymphoma if the inflammatory background is positive?

  • Bilobed nucleus
  • Presence of Reed-Sternberg cells (correct)
  • Prominent eosinophilic nucleoli
  • All of the above

    • Nodular lymphocyte predominant Hodgkin's Disease has poor prognosis.

    False

    What is the most common subtype of Classical Hodgkin's Disease worldwide?

    Nodular sclerosis (NS)

    The subtype of Hodgkin's Disease associated with 100% EBV involvement is ________.

    <p>lymphocyte depleted (LD)</p> Signup and view all the answers

    Match the following Hodgkin's Disease subtypes with their characteristics:

    <p>Lymphocyte Rich = 20% EBV involvement Mixed Cellularity = 70% EBV involvement Lymphocyte Depleted = 100% EBV involvement Nodular Sclerosis = 40% EBV involvement</p> Signup and view all the answers

    Which of the following markers differentiates Activated B-cell DLBCL from Germinal Center DLBCL?

    <p>MUM-1 expression +ve</p> Signup and view all the answers

    All presentations of Activated B-cell DLBCL have a good prognosis.

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

    What is the treatment regimen typically used for Non-Hodgkin's Lymphoma?

    <p>R-CHOP</p> Signup and view all the answers

    The presence of _____ and aberrant BCL-2 is a marker for poor prognosis in DLBCL.

    <p>dysregulated BCL-6</p> Signup and view all the answers

    Match the terms with their correct definitions:

    <p>Double hit = c-myc rearrangement + rearrangement of BCL-2/BCL-6 Triple hit = Rearrangement of c-myc + BCL-2 + BCL-6 GCB DLBCL = Good prognosis Activated B-cell DLBCL = Bad prognosis</p> Signup and view all the answers

    What type of lymphoma is associated with centrocytes?

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

    The light zone of a lymph node is where B cells mature and proliferate.

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

    What are the two types of lymphomas associated with centroblasts?

    <p>Diffuse large B-cell lymphoma and Burkitt's lymphoma</p> Signup and view all the answers

    Memory B cells are ___ differentiated B cells.

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

    Match the lymphoid tumors with their cell/site of origin:

    <p>Follicular lymphoma = Centrocytes Mantle-cell lymphoma = Pre-GC Marginal zone lymphoma = Post-GC Multiple myeloma = Plasma cells</p> Signup and view all the answers

    What is the most common immunoglobulin produced in plasma cell disorders?

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

    Monoclonal proliferation of plasma cells is characterized by the presence of multiple homogeneous immunoglobulins.

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

    What is a common marker found on monoclonal plasma cells?

    <p>CD138</p> Signup and view all the answers

    In plasma cell disorders, the ratio of ___ to ___ is typically noted as κ > λ.

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

    Match the following plasma cell disorders with their corresponding characteristics:

    <p>Multiple myeloma = Most prevalent plasma cell disorder Waldenstrom's macroglobulinemia = Type of lymphoma with monoclonal IgM production AL-Amyloidosis = Condition characterized by abnormal protein deposits Heavy chain disease = Characterized by the production of abnormal heavy chains only</p> Signup and view all the answers

    What is the most common translocation associated with Burkitt's lymphoma?

    <p>t(8;14)</p> Signup and view all the answers

    Burkitt's lymphoma is characterized by a 'Starry Sky Appearance' under the microscope.

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

    Name one type of Burkitt's lymphoma that is EBV positive.

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

    Burkitt's lymphoma has a high level of the ________ protein due to c-myc dysregulation.

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

    Match the following types of Burkitt's lymphoma with their characteristics:

    <p>Endemic = Children, EBV positive, Jaw/maxillary mass Sporadic = Adults, EBV negative, Abdominal mass HIV associated = Occurs in immunodeficient patients</p> Signup and view all the answers

    What is the survival rate for early stage Hodgkin's Lymphoma (favorable)?

    <blockquote> <p>90%</p> </blockquote> Signup and view all the answers

    All B-cells either survive or undergo apoptosis related to POL gene aberration or Hodgkin's disease.

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

    What age group is commonly affected by Non-Hodgkin's Lymphoma?

    <p>15-34 years</p> Signup and view all the answers

    The most common extranodal site involved in Non-Hodgkin's Lymphoma is the ________.

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

    Which type of lymphoma is characterized by CD30+ and the presence of hallmark cells?

    <p>Anaplastic large cell lymphoma</p> Signup and view all the answers

    Match the features with Hodgkin's Lymphoma (HL) and Non-Hodgkin's Lymphoma (NHL):

    <p>Bimodal age distribution = HL Presenting age of 15-34 years = NHL Extracnodal involvement = NHL High cure rates in early stages = HL</p> Signup and view all the answers

    Extranodal NK T-lymphoma presents with a nasal mass and has a poor prognosis.

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

    Which medication is associated with cardiotoxicity?

    <p>Adriamycin (Doxorubicin)</p> Signup and view all the answers

    Name two clinical features associated with Hodgkin's disease.

    <p>Diffuse painless rubbery firm lymphadenopathy, Alcohol induced increase in node size.</p> Signup and view all the answers

    The type of lymphoma commonly found in HIV positive patients and characterized by extensive B symptoms is _____ cellularity.

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

    The Deauville score of 1-2 indicates an early unfavorable response to chemotherapy.

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

    Match the following types of lymphomas with their characteristics:

    <p>Endemic Burkitt's lymphoma = Associated with EBV in endemic regions Post-transplant lymphoma = Occurs in immunocompromised patients post-organ transplant Primary CNS lymphoma = Malignant tumor in the central nervous system Nodular sclerosis = Most common subtype in young females with mediastinal involvement</p> Signup and view all the answers

    What additional treatment is indicated for refractory or advanced disease?

    <p>Brentuximab (Anti-CD30)</p> Signup and view all the answers

    In the classical HD protocol, the treatment involves cycles of ___ chemotherapy.

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

    Match the chemotherapy drug with its associated side effect:

    <p>Adriamycin (Doxorubicin) = Cardiotoxicity Bleomycin = Lung fibrosis Vinblastine = Peripheral neuropathy Dacarbazine = No significant side effect listed</p> Signup and view all the answers

    What level of m-protein is associated with Monoclonal gammopathy of undetermined significance (MGUS)?

    <p>11 mg/dL</p> Signup and view all the answers

    Anemia is classified as a myeloma defining event (MDE) when the hemoglobin is below 3 g/dL.

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

    What assay is used to identify non-secretory myeloma with a sensitivity of 97%?

    <p>Serum free light chain assay (SFLC)</p> Signup and view all the answers

    In cases of renal failure, the serum creatinine level is greater than ______ mg/dL.

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

    Match the following events with their corresponding measurements:

    <p>MGUS = 11 mg/dL m-protein Renal failure = S. Creatinine &gt;2 mg/dL Anemia = Hb 3g/dL Sensitivity of SFLC = 97%</p> Signup and view all the answers

    What is the investigation of choice for staging Hodgkin's Disease?

    <p>Full thickness excision biopsy</p> Signup and view all the answers

    Stage IV of lymphoma indicates localized disease in a single lymph node region.

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

    What is the significance of a PET scan in the context of lymphoma?

    <p>It is the single most valuable test for staging.</p> Signup and view all the answers

    Age greater than 60 and positive B symptoms are considered ______ in Hodgkin's Disease prognosis.

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

    Match the stages of lymphoma with their descriptions:

    <p>Stage I = Localized disease; single lymph node region or single organ Stage II = Two or more lymph node regions on the same side of the diaphragm Stage III = Two or more lymph node regions above and below the diaphragm Stage IV = Widespread disease; multiple organs, with or without lymph node involvement</p> Signup and view all the answers

    Study Notes

    Hodgkin’s Disease

    • Reed-Sternberg (RS) cells are a hallmark of Hodgkin’s Lymphoma (HL)
    • RS cells have bilobed nucleus, prominent eosinophilic nucleoli, and a clear space between the nuclear membrane and nucleoli
    • RS cells are only specific for HL if there is inflammatory background
    • Nodular lymphocyte predominant HD is a benign entity and has excellent prognosis, usually involves axillary lymph node
    • Classical HD subtypes:
      • Lymphocyte rich (LR)
      • Lymphocyte depleted (LD)
      • Nodular sclerosis (NS): Most common subtype worldwide
      • Mixed cellularity (mc): Most common subtype in India
    • CD15, CD30, CD20, and CD45 are present in both Classical and Nodular subtypes of HD
    • Surface immunoglobulin is negative in Classical HD and positive in Nodular HD
    • EBV involvement percentages as per the 100/70/40/20 rule for EBV involvement:
      • LD: 100%
      • mc: 70%
      • NS: 40%
      • LR: 20%

    Histology

    • Nodular HD: Popcorn RS cell/ Lymphocyte histiocyte cells are present and RS cells are absent
    • Classical HD subtypes: Classical RS cells are present in mc, Lacunar RS cells are present in NS, and Paucity of RS cells are present in LR

    Physiology

    • Dark Zone (Centroblasts): Located in the center of the lymph node.
    • Germinal Center (GC): A site where B cells mature and proliferate.
    • Light Zone (Centrocytes): Located close to the dark zone.
    • Post-GC: Cells leaving the germinal center.
    • Memory B-cells: Mature differentiated B cells.

    Pathology

    • Cell/site of origin of Lymphoid Tumors
      • Centrocytes: Follicular lymphoma
      • Pre-GC: mantle-cell lymphoma (MCL)
      • Centroblasts:
        • Diffuse large B-cell lymphoma (DLBCL)
        • Burkitt's lymphoma
      • Post-GC: marginal zone lymphoma (MZL)
      • Plasmablasts: Activated B-cell Diffuse B-cell Lymphoma
      • Plasma cells: multiple myeloma
      • Memory B cells:
        • Small lymphocytic lymphoma (SLL)
        • Waldenström's macroglobulinemia
        • Hairy cell leukemia
    • Pathogenesis
      • Upregulation of NK-kB seen.
      • m>F (likely means male to female ratio)

    Non-Hodgkin's Lymphoma (NHL)

    • GCB (Germinal Center B-cell lymphoma): CD10, CD23, BCL-6 +ve, good prognosis
    • Activated B-cell DLBCL: Cells of origin are Plasmablasts.
      • Markers:
        • CD10-ve, BCL-6 +ve, MUM-1 expression +ve
        • CD10-ve, BCL-6 -ve
        • Presentations:
          • CNS lymphoma
          • Mediastinal lymphoma
          • Organ transplant lymphoma
          • Body cavity lymphoma
          • HIV-defining illness
      • Poor prognosis
    • Other presentations are noted as "ABC-DLBCL unless proven otherwise"
    • Dysregulated BCL-6 > Aberrant BCL-2 (t(14;18))
    • In follicular lymphoma the opposite is true (Aberrant BCL-2 > Dysregulated BCL-6)
    • Double hit: c-myc rearrangement + rearrangement of BCL-2/BCL-6
    • Triple hit: Rearrangement of c-myc + BCL-2 + BCL-6
      • Prognosis: Bad
    • Treatment: R-CHOP (Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Oncovin (Vincristine), Prednisolone)

    Burkitt's Lymphoma

    • A type of childhood lymphoma (30% of childhood NHL)
    • Can involve the Central Nervous System (CNS)
    • Chemosensitive
    • CD10 and BCL-6 are positive
    • Pathophysiology
      • c-myc dysregulation on chromosome 8
      • High levels of myc protein
      • Highest proliferative index (Ki67) among NHL
    • Translocation:
      • t(8;14): m/c
      • t(8;2)
      • t(8;22)
    • Types:
      • Endemic:
        • Children
        • EBV positive
        • Jaw/maxillary mass
      • Sporadic:
        • Adults
        • EBV negative
        • Abdominal mass
        • Bone marrow involvement (>30% cases)
      • HIV associated:
    • Treatment:*
    • CODOX-M/IVAC regime
    • Doesn't respond to conventional chemotherapy
    • Lymphomas with bone marrow involvement:
      • Burkitt's lymphoma
      • Mantle cell lymphoma
      • DLBCL (20%)
    • Microscopic Appearance: Starry Sky Appearance -> Stars -> macrophages, Sky -> Tumor cells.

    Plasma Cell Disorders

    • Physiology of Plasma Cells
      • Germinal center B-cell → marginal zone → memory B-cell
      • Activated B-cell (ABC plasma blast)
        • Plasma cell: CD19, CD38, CD138
        • (Polyclonal)
      • IgG, IgM, IgA, both κ and λ
    • Plasma Cell Disorder
      • Monoclonal Proliferation of Plasma Cells (m-protein):
        • Monoclonal plasma cell:
          • CD19+
          • CD38+
          • CD138+
          • CD45
        • κ > λ
        • IgG > IgA
        • m/c: IgGK
      • Expansion of a clone of Ig secreting, heavy chain class switched, terminally differentiated B cells that typically secrete a single homogeneous (monoclonal) immunoglobulin called a paraprotein or m-protein.
    • Types:
      • Multiple myeloma
      • Waldenstrom's macroglobulinemia
      • AL-Amyloidosis
      • Heavy chain disease

    Hematology

    • EBV associated lymphomas
      • HD & NHL:
        • B cell type:
          • Endemic Burkitt's lymphoma
          • Post-transplant lymphoma
          • Primary CNS lymphoma
        • T cell type:
          • Extranodal NK T-lymphoma
            • Very poor prognosis
            • Presents with nasal mass
    • Anaplastic large cell lymphoma:
      • CD30+
      • Histology: Hallmark cells, flower cells

    Clinical Features of HL

    • Diffuse painless rubbery firm lymphadenopathy:
      • Site: Posterior cervical region (60-80%) > Supraclavicular > Axillary (seen in lymphocyte-predominant HD)
    • 8 Symptoms (30%-40%): Weight loss, night sweats, fever
      • m > F
      • 8 Symptoms are often present
      • Alcohol induced ↑ size in lymph node
    • Paraneoplastic manifestations:
      • Subacute cerebellar degeneration
      • Cholangitis
      • Vanishing bile duct syndrome
    • Other Manifestations:
      • Unexplained pruritus
      • Pel-Ebstein fever: Diagnostic
      • Nodular sclerosis:
        • Mediastinal node
        • Young females
        • SVC obstruction
        • Pleural effusion
      • Mixed cellularity:
        • Common in HIV+ve patients
        • Seen in LD also
        • 70% EBV+ve
        • Extensive B symptoms
        • Elderly, children
        • Classic RS cells and mononuclear cells
    • Images:
      • Popcorn RS cell: Image describing the appearance of popcorn RS cells.
      • Lacunar cells: Image showing lacunar cells
      • Nodular sclerosis: Image displaying nodular sclerosis
      • Mixed cellularity: Image depicting mixed cellularity
      • Several other images (not described) of histological slides are not included in this summary.

    B-cell Development and Outcomes

    • B-cells move to dark zone and undergo multiple somatic hypermutations and affinity maturation.
    • Some B-cells survive.
    • Others undergo apoptosis related to POL gene aberration or Hodgkin's disease.

    Differences between HL and NHL
    Survival Rates
    Age of Presentation Bimodal, peak ~760 yrs
    Lymphoid Tissue Involved
    CNS Involvement
    B symptoms
    Contiguous Involvement (lymph nodes share the same border)
    EBV Involvement +
    • Pel-Ebstein fever: Low-grade irregular fever, spikes gradually. Cyclical fever. Afebrile.
    • Cure Rates:
      • Early stage (I/II) favorable: >90%
      • Early stage (I/II) unfavorable: ~85%
      • Advanced (III/IV): ~80%

    Treatment Protocol for Hematology

    • mc: Poor response to chemotherapy
    • LD: Poor response to chemotherapy
    • NS: Very good response to chemotherapy
    • LR: Excellent response to chemotherapy

    Chemotherapy Regimen: ABVD
    Drug
    Adriamycin (Doxorubicin)
    Bleomycin
    Vinblastine
    Dacarbazine

    Protocol 1: Classical HD

    • Cycle of ABVD Chemotherapy
    • Interim PET scan: to determine the score using the Deauville 5 point scale
    • Possible Outcomes and Subsequent Actions:
      • Early favorable (PET-ve, Deauville score 1-2): 2 more cycles of chemotherapy.
      • Early unfavorable (Deauville score 1-2 after initial cycles): 3 more cycles of chemotherapy.
      • Interim PET with Deauville score of 3, 4, or 5: Salvage chemotherapy along with autologous stem cell transplant.
      • Advanced disease: 4 more chemotherapy cycles.
      • If patient has refractory or advanced disease: BEACOPP is used
    • Involved site/field radiotherapy:
      • Not used in LPHD
      • Can be given with ABVD
    • Additional Considerations (for relapses or refractory cases):
      • Brentuximab (Anti-CD30): Indicated for refractory or advanced disease.

    Monoclonal Gammopathies

    Condition m-protein (serum) Bone marrow plasma cells Myeloma defining events (MDE)
    Monoclonal gammopathy of undetermined significance (MGUS) 11 mg/dL < 10% None.
    Smoldering multiple myeloma < 3 g/dL 10-60% None.
    Multiple myeloma > 3 g/dL > 10% At least 1 of the following: * Renal failure: S.Creatinine >2 mg/dL * Anemia: Hb 3g/dL * Serum Immuno-electrophoresis (IEP): urine also used * Serum free light chain assay (SFLC): * Picks up non-secretory myeloma. * 97% sensitivity

    Richter Transformation

    • Low grade lymphoma:
      • Eg: SLL
      • Follicular lymphoma
    • Transforms to: High grade lymphoma (DLBCL).

    Hodgkin's Disease Staging

    • Investigation of choice: Full thickness excision biopsy.
    • Stages:
      Stage Description
      Stage I Localized disease; single lymph node region or single organ
      Stage II Two or more lymph node regions on the same side of the diaphragm
      Stage III Two or more lymph node regions above and below the diaphragm
      Stage IV Widespread disease; multiple organs, with or without lymph node involvement
    • Disease Progression:
      • Early disease
      • Late/Advanced disease
    • Favorable prognosis:
      • Age > 60
      • B symptoms +ve
      • Bulky nodes (>10cm)
      • 24 extranodal sites
      • ESR 250 mm/hr
    • Unfavorable prognosis
      • Age > 60
      • B symptoms +ve
      • Bulky nodes (>10cm)
      • 24 extranodal sites

    PET Scan

    • Single most valuable test for staging
    • Uses FDG (Fluoro deoxyglucose)

    Other Paraneoplastic Manifestations

    • ↑↑ LDH
    • Hypercalcemia
    • Hypoglycemia
    • Hyponatremia

    Beta-2 microglobulin

    • Indicates poor prognosis in CLL, HD, MM.

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