Clinical Classification of Lymphocyte Disorders

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  1. Name 3 major types of lymphocyte disorders.
    • —1. Inborn errors of lymphocyte metabolism—
    • 2. Immune dysfunction
    • 3. —“Acquired” Disorders
  2. Name 3 types of localized lymphocyte disorders and some examples.
    • 1. Local infection: pyogenic infection like pharyngitis, dental abscess, otitis media, actinomyces, viral infection, cat scratch fever, lymphogranuloma venereum, tuberculosis
    • 2. Lymphoma: Hodgkin lymphoma, non-Hodgkin lymphoma
    • 3. Carcinoma
  3. Name 4 categories of generalized lymphatic disorders with examples.
    • 1. Infection: viral like mononucleosis, measles, rubell, viral hepatitis, HIV; bacterial like syphilis, brucellosis, tuberculosis, Salmonella,
    • bacterial endocarditis; fungal like histoplasmosis; protozoal like toxoplasmosis
    • 2. Non-infectious inflammatory diseases like sarcoidosis, rheumatoid arthritis, SLE, other connective tissue diseases, serum sickness
    • 3. Malignant like leukemias, lymphomas (Hodgkin/non Hodgkin), secondary carcinoma (rare), angioimmunoblastic lymphadenopathy
    • 4. Miscellaneous like sinus histocytosis with massive lymphadenopathy (Rosai-Dorfman), reaction to drugs and chemicals, hyperthyroidism
  4. Describe the inborn errors of lymphocyte metabolism. 
    • —Internal defects in lymphoid cells that result in functional abnormalities of marrow-derived B-Cells and thymic derived lymphocytes
    • —Impairment of both humoral and cellular immunity
    • —Considered a primary disorder
    • —Errors in both metabolism and receptor/ ligand expression
  5. Name 11 primary lymphocyte disorders.
    • 1.—B-Lymphocyte Deficiency
    • 2.—Agammaglobulinemia—
    • 3.Selective agammaglobulinemia—
    • 4.Hyperimmunoglobulins of A, E, D.
    • 5.—Immunodeficiency
    • 6.—X-linked immunodeficiency
    • 7.—T-lymphocyte Dysfunction
    • —8.Lymphocyte Function Antigen 1 deficiency—
    • 9.Thymic Aplasia (DiGeorge Syndrome)—10.Thymic Hypoplasia
    • 11.—Wiskott-Aldrich syndrome
  6. Name 3 lymphocyte disorders with combined
    T- and B-Cell dysfunction.
    • 1.—Ataxia-telangietasia
    • 2.—Defective expression of major histocompatability antigens
    • 3.—Pyridoxine deficiency (related to lymphocytes being formed)
  7. Name 5 acquired lymphocyte disorders.
    1. Acquired Immunodeficiency Syndrome (AIDS)

    • Reactive Lymphocytosis or plasmacytosis:
    • —2.Bordetella pertussis lymphocytosis—
    • 3.Cytomegalovirus mononucleosis
    • 4.—Ebstein-Barr Virus Mononucleosis—
    • 5.Drug-induced lymphocytosis
  8. Name 6 lymphocyte disorders that have a T-Cell dysfunction and are associated with systemic disease.
    • —1.B-Cell Chronic Lymphocytic Leukemia—
    • 2.Hodgkin lymphoma
    • 3.—Leprosy—
    • 4.Lupus Erythematosus
    • 5.—Rheumatoid Arthritis—
    • 6.Sarcoidosis
  9. Quantitiative: What lab result indicates lymphocytosis? TQ
    —Absolute lymphocyte count exceeding 4 x 10^9/L (4000/mL) TEST QUESTION calculate absolute lymph count and know its lymphocytosis
  10. In a patient where lymphocytosis is suspected, what should blood smears be evaluated for?
    reactive lymphocytes, smudge cells
  11. What lab test can distinguish B vs T-Cells by characterization of surface markers?
    Flow cytometry
  12. What is primary lymphocytosis?
    • —Increase in the absolute count of lymphocytes as a lymphoproliferative disorder
    • —Usually seen through clonal expansion of B- or T-Lymphocytes in the bone marrow—
    • B-Cells, T-Cells, and natural killers cells are fully differentiated, other lymphocytes are less differentiated—
    • Lymphocyte counts will rise over time
  13. Describe Monoclonal (produces 1 Ab) B-Cell Lymphocytosis
    • —An expanded population of monoclonal B-Cells without other associated clinical signs or symptoms
    • —Similar to patients with monoclonal gammopathy, but different
    • —Monoclonal B-Cell lymphocytosis may progress to a neoplastic lymphoproliferative disorder
  14. Describe 4 points of Persistent Polyclonal Lymphocytosis of B-Lymphocytes (PPBL).
    • —1.Express many immunoglobulins
    • 2.—Increased numbers of kappa and lambda expressing B-Cells
    • 3.—May emerge into one predominate clonal immunoglobulin—
    • 4.Associated with EBV
  15. Name 7 disorders involving reactive lymphocytosis due to infection.
    • 1.—EBV
    • 2.—CMV
    • 3.—Toxoplasma gondii
    • 4.—HIV
    • 5.—Herpes Simplex Virus II
    • 6.—Adenovirus
    • 7.—Hepatitis
  16. Name 6 things that can lead to chronic lymphocytosis.
    • 1.—Autoimmune disorders
    • 2.—Cancer
    • 3.—Cigarette smoking: particulate matter in cigarrette smoke causes immune system rxn
    • 4.—Chronic inflammation
    • 5.—Hyposplenism—
    • 6.Post-splenectomy
  17. Name 6 things that can lead to stress-induced lymphocytosis (acute)
    • —1.Cardiac failure
    • 2.—Septic shock
    • 3.—Drug-induced
    • 4.—Major surgery
    • 5.—Sickle cell crisis
    • 6.—Trauma
  18. Name 4 indications of lymphocytopenia.
    • —1.Absolute lymphocyte count less that 1x109/L or 1000/mL
    • 2.—80% of blood lymphocytes are T-lympocytes
    • 3.—2/3 of T-lymphocytes are T-Helper Cells (CD4+)
    • 4.—Lymphocytopenia may also be defined by a decrease in the overall T-Helper Cell Count
  19. Name 4 disorders associated with inherited lymphocytopenia.
    • —Congenital immunodeficiency diseases:
    •     1.—Aplasia of lymphopoetic stem cells
    •     2.—Adenosine deaminase deficiency—
    • 3.Wiskott Aldrich syndrome 
    • —4.Dwarfism
  20. Name 10 disorders associated with acquired lymphocytopenia.
    • —1.Aplastic anemia
    • —Infectious bacterial and viral diseases:
    •        2.—AIDS
    •        3.Influenza—
    • 4.Thermal injury
    • 5.—Renal or marrow transplant
    • 6.—Radiation— 
    •        Destruction of cells through cutaneous  tissue
    • 7.—Usually due to a primary disorder of T-Helper Cells— 
    •       Impaired proliferation—
    • 8.Dietary Zinc deficiency—
    • 9.Excessive intake of alcohol
    • 10.—Severe redistribution of T-Cells to the tissues during trauma
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Clinical Classification of Lymphocyte Disorders
2013-02-10 19:45:42
hematology exam two

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