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70 Cards in this Set
- Front
- Back
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Errors in lymphocytic metabolism are considered a _____ disorder and impair both the ________ and ________ immunity
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primary, humoral, cellular
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Major histocompatibility complex
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self versus non-self recognition, especially tissue antigen
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Acquired lymphocytic disorders
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AIDS, reactive lymphocytosis or plasmacytosis (Bordetella, CMV, Epstein-Barr, drug-induced)
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Systemic diseases associated with T-cell dysfunction
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B-cell chronic lymphocytic leukemia, Hodgkin lymphoma, leprosy, lupus erythematosus, rheumatoid arthritis, sarcoidosis
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Lymphocytosis
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absolute lymphocyte count >4,000/microliter
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How can B-cells and T-cells be distinguished?
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By characterization of surface markers using flow cytometry
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M-spike
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monoclonal B-cell lymphocytosis
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Monoclonal B-cell lymphocytosis may progress to ___________
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a neoplastic lymphoproliferative disorder
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Persistent polyclonal lymphocytosis of B-lymphocytes is associated with what virus?
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Epstein-Barr
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Chronic lymphocytosis may be associated with:
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autoimmune disorders, cancer, cigarette smoking, chronic inflammation, hyposplenism, post-splentectomy
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Lymphocytopenia is a lymphocyte count less than ________
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1000/microliter
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What is the major type of lymphocyte in the body?
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T-lymphocyte (80%)
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2/3 of lymphocytes are ________
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T-helper cells (CD4+)
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Acquired lymphocytopenia is associated with
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marrow transplant, radiation, aplastic anemia, AIDS, influenza, thermal injury, bacterial or viral infection
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Plasma cell disorders are conditions in which __________
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monoclonal plasma cells replicate excessively, overproducing one type of antibody-like molecule
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Monoclonal gammopathies of undetermined significance
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plasma cells are abnormal but not cancerous, stable for years and does not initially require treatment, may progress to multiple myeloma (20-30%)
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Multiple myeloma
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plasma cell cancer, clone of abnormal plasma cells multiplies, tumors in bone marrow, large quantity of abnormal antibodies accumulate in blood or urine
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Immunoglobulin deficiencies are associated with a deficiency of what cell?
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B lymphocytes
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X-Linked agammaglobulinemia
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- inherited disorder
- inability to produce IgG - B lymphs and plasma cells absent from lymph nodes, spleen, intestine and marrow - Detected in second year of life; pyogenic infections common |
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Laboratory features of X-linked agammaglobulinemia
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<100mg/dL of IgG, undetectable IgA, IgD, IgM, IgE
- treated with gamma globulin infusion |
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Wiskott-Aldrich syndrome:
genetics and symptoms |
x-linked recessive
eczema, thrombocytopenia, recurrent infection - boys rarely survive first decade of life |
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AIDS laboratory symptoms
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CD4 < 200/uL
PCR during acute phase CD4/CD8 ratio |
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Characteristics of HIV-1
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RNA retrovirus that integrates its genome into genome of infected T cells; characterized by long periods of latency with subsequent onset of symptoms
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Transmission of HIV
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Sexual, parenteral drug use, infected blood products, and vertical
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HIV infects cells containing which surface membrane protein
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CD4+ cells: T-helpers, monocytes, follicular dendritic, megakaryocytes and langerhans' cells
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Pathogenesis of HIV
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- depletion of CD4+ T-cells
- defects in B-cell immunity (inc. risk of autoimmune phenomena) - defects in natural killer cells |
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Hematologic abnormalities of HIV
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- anemia
- neutropenia - thrombocytopenia (ie. ITP) - Lymphoma - Malignancies |
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Anemia in AIDS is due to
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- decreased production (ACD, blunted EPO response, infection, IDA)
- Ineffective production (folic acid def., B12 def., gastric pathology - pernicious) - increased destruction (hemophagocytosis, TTP, DIC and G6PD deficiency due to HIV drugs) |
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Neutropenia is seen in AIDS due to
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- decrease in CFU-GM and G-CSF as well as defective function of opsonization in neutrophils and macrophages
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Thrombocytopenia is seen in AIDS because?
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- cross reactive antibody against GP160/120 and GPIIb/IIIa
- immune destruction of platelets - direct infection of megakaryocytes by HIV |
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how is HIV related to malignant disorders?
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- seen in 40% of HIV patients
- Karposi's Sarcoma - B-cell lymphoma - Chronic use of immunosuppresive drugs causes disregulated expression of cytokines |
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The French-American-British classification arranges _________ into labeled categories using ______ and ______
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acute leukemias, cell maturity, lineage
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Type of myelodysplastic syndromes
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1. Refractory anemia
2. Refractory anemia with ringed sideroblasts 3. Refractory anemia with excess blasts 4. Refractory anemia with excess blasts in transformation 5. Chronic myelomonocytic leukemia |
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Types of myeloproliferative disorders
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1. Chronic myelogenous leukemia
2. Polycythemia vera 3. Essential thrombocythemia |
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World Health Organization Neoplasms Classification is based on which lineages?
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myeloid, lymphoid, mast cell, histiocytic
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Leukemia is characterized by the replacement of normal cells in the ________ with _______ cells
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bone marrow, neoplastic
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Philadelphia chromosome
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Translocation of chromosomes 9 and 22, highly correlated (90-95%) with chronic myelogenous leukemia
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Define myelodysplastic syndrome
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Primary, neoplastic, pluripotential stem cell disorder characterized by one or more peripheral blood cytopenias and prominent maturation abnormalities in the bone marrow
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M6
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Erythroleukemia
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M7
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Acute Megakaryoblastic Leukemia
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M0
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Acute myeloblastic leukemia without cytologic maturation
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M1
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Acute myeloblastic leukemia with minimal cytological maturation
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M2
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Acute myeloblastic leukemia with cytological maturation
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Cytochemical stains are useful for differentiating ___________ from leukemia of __________ origin.
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acute myelogenous leukemia, lymphoid
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Sudan Black B stains _____ and ______
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phospholipids and sterols
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Lymphocytes and lymphoblasts are Sudan Black B _________.
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negative
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Peroxidase stains stain ________.
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azurophilic granules
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Acid phosphatase cytochemical stain is useful for confirming a diagnosis of ______
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Hairy-cell leukemia
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AML requires what treatment intensity?
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Very aggressive due to the rapid onset of this disorder
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AML usually occurs in children (T/F)
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False, AML is a disease of adulthood
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Ulceration of mucous membranes is a common finding in _________.
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Acute myelogenous leukemia
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Hyposegmentation is seen in many of the neutrophils in what leukemias? This is known as __________.
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M1 and M2, pseudo-Pelger-Huet anomaly.
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What are Auer rods?
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primary granules that are fused together.
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M3
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Promyelocytic leukemia
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Auer rods are found in most patients with ________.
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Promyelocytic leukemia (M3).
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Hypergranular M3 leukemia may result in what coagulation problem?
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DIC due to release of procoagulant material from abnormal promyelocytes.
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M4 leukemia is characterized by what cells?
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Predominance of both monocyte and granulocyte precursor cells (both in bone marrow and peripheral blood)
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M5
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Monoblastic leukemia
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Erythroleukemia is also known as __________.
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Di Guglielmo's syndrome
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L2 is the most common lymphocytic leukemia for what age group?
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adults
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Neutrophilia
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increase in absolute neutrophil count that is the result of a physiologic or pathologic process (usually greater than 6,000 per microliter)
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Neutrophilia is frequently accompanied by a ______ shift, which indicates more _______ cells
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left, immature
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Three major causes of neutrophilia
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infection, inflammation, toxic chemicals/drugs
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Physiologic neutrophilia is typically a response to _______
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therapy
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A pseudo-neutrophilia is caused by ______, and is ______ in nature.
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physical or emotional stress, transient
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Leukemoid reaction
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extreme neutrophilic reaction to severe infection or necrotizing tissue, WBC > 25 x 10^9/L
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A leukemoid reaction mimics what leukemia?
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CML
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______ is the most common type of leukopenia
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Neutropenia
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Agranulocytosis
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term used to describe extreme neutropenia (<0.3-0.5 x 10^9/L)
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Increased neutrophil destruction may occur due to:
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infection, immune reaction, sequestration
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