Clinical Classification of Lymphocyte Disorders
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Name 3 major types of lymphocyte disorders.
- 1. Inborn errors of lymphocyte metabolism
- 2. Immune dysfunction
- 3. “Acquired” Disorders
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
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
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
Name 11 primary lymphocyte disorders.
- 1.B-Lymphocyte Deficiency
- 3.Selective agammaglobulinemia
- 4.Hyperimmunoglobulins of A, E, D.
- 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
Name 3 lymphocyte disorders with combined
T- and B-Cell dysfunction.
- 2.Defective expression of major histocompatability antigens
- 3.Pyridoxine deficiency (related to lymphocytes being formed)
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
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
- 4.Lupus Erythematosus
- 5.Rheumatoid Arthritis
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
In a patient where lymphocytosis is suspected, what should blood smears be evaluated for?
reactive lymphocytes, smudge cells
What lab test can distinguish B vs T-Cells by characterization of surface markers?
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
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
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
Name 7 disorders involving reactive lymphocytosis due to infection.
- 3.Toxoplasma gondii
- 5.Herpes Simplex Virus II
Name 6 things that can lead to chronic lymphocytosis.
- 1.Autoimmune disorders
- 3.Cigarette smoking: particulate matter in cigarrette smoke causes immune system rxn
- 4.Chronic inflammation
Name 6 things that can lead to stress-induced lymphocytosis (acute)
- 1.Cardiac failure
- 2.Septic shock
- 4.Major surgery
- 5.Sickle cell crisis
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
Name 4 disorders associated with inherited lymphocytopenia.
- Congenital immunodeficiency diseases:
- 1.Aplasia of lymphopoetic stem cells
- 2.Adenosine deaminase deficiency
- 3.Wiskott Aldrich syndrome
Name 10 disorders associated with acquired lymphocytopenia.
- 1.Aplastic anemia
- Infectious bacterial and viral diseases:
- 4.Thermal injury
- 5.Renal or marrow transplant
- 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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