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70 Cards in this Set

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