Term
|
Definition
| Malignant proliferations of WBC precursor cells within the bone marrow that spread to the blood and other organs |
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Term
| 4 major types of leukemia |
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Definition
acute lymphoblastic leukemia (ALL)
acute myelogenous leukemia (AML)
chronic lymphocytic leukemia (CLL)
chronic myelogenous leukemia (CML) |
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Term
| Cell types in classification of Leukemias |
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Definition
1.lymphoid (derived from lymphocytic precursor cells) 2. myeloid (derived from neutrophilic,monocytic, RBC or platelet precursor cells)
-most commonly from neutrophilic and monocytic precursor cells |
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Term
| Developmental maturity of cells in classification of Leukemias |
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Definition
| 1. Acute leukemias:predominance of BLASTS (Blast cells) - most immature cells with limited differentiation 2. Chronic leukemias:predominance of most mature, differentiated cells. e.g. mature lymphocytes; neutrophils |
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Term
|
Definition
| Maturation of blood cells in bone marrow is from extreme left (most immature, undifferentiated cells called blasts ) toward the extreme right ( most mature, differentiated cells e.g. neutrophils ) |
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Term
|
Definition
| Acute lymphoblastic leukemia (ALL) Acute myelogenous leukemia (AML) |
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Term
| Acute lymphoblastic leukemia (ALL) |
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Definition
1. Incidence: Most common leukemia in children; also occurs in adults Most common pediatric cancer; peak age 2-10
2.Prognosis: many cures (up to 70% children; 50% adults) |
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Term
| Acute myelogenous leukemia (AML) |
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Definition
| 1.Incidence: median age 50 yrs; can occur in children 2.Prognosis: worse than ALL (up to 60% cure with bone marrow transplant; up to 40% with chemotherapy alone ) |
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Term
| CLINICAL PRESENTATION OF ACUTE LEUKEMIAS-(Labs) |
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Definition
| Blood: a. Blasts not present in a minority of cases b. Pancytopenia (anemia;thrombocytopenia;leukopenia) may be present due to replacement of bone marrow with blasts c. WBC count may be variable depending on the number of blasts in the blood d. AML: very large # blasts may be present -WBC count >100K *** THINK OF ACUTE LEUKEMIA WHEN BLASTS ARE REPORTED ON CBC AND GET A HEMATOLOGY CONSULT RIGHT AWAY*** |
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Term
| CLINICAL PRESENTATION OF ACUTE LEUKEMIAS-(Signs & Symptoms) |
|
Definition
1.Typically acute or abrupt onset
2.Both AML and ALL:
-Bone pain (due to replacement of bone marrow with blasts)
-Hepatosplenomegaly
-Signs of anemia: pallor; weakness
-Signs of thrombocytopenia: easy bruisability; epistaxis (nose bleed); gum bleeding
-Signs of leukopenia and neutropenia:infection
-Central nervous system involvement(CSF)
-Involvement of testes may occur
-May present as mediastinal mass
-Lymphadenopathy (enlargement of lymph nodes in 50% cases) |
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Term
|
Definition
CHRONIC MYELOGENOUS LEUKEMIA (CML)
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL) |
|
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Term
CHRONIC MYELOGENOUS LEUKEMIA (CML)
INCIDENCE |
|
Definition
| INCIDENCE: median age at presentation- 50 years,can occur in children |
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Term
CHRONIC MYELOGENOUS LEUKEMIA (CML)
CLINICAL PRESENTATION |
|
Definition
Easy fatiguability and weight loss;
often asymptomatic
Splenomegaly in most patients; may be extreme
-Signs of splenomegaly:left upper quadrant pain; early satiety |
|
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Term
CHRONIC MYELOGENOUS LEUKEMIA (CML)
BLOOD LABS |
|
Definition
- very high WBC count (usually > 30,000/uL)
Predominance of neutrophils along with neutrophil precursor cells ( bands; metamyelocytes; myelocytes; promyelocytes )
Insignificant # of blasts (most immature cells)
Anemia and thrombocytopenia may be present |
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Term
| CHRONIC MYELOGENOUS LEUKEMIA (CML) BONE MARROW LABS |
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Definition
| similar findings as in blood Decreased leukocyte alkaline phosphatase ( LAP ) score: Leukocyte alkaline phosphatase ( LAP ) is an enzyme stained in neutrophils and evaluated on a peripheral blood smear. The quantity of staining of LAP on the smear determines the LAP score (some labs do not perform this test at this time ) A characteristic of neutrophils in CML is decreased quantity of the enzyme LAP, and therefore decreased staining for LAP -> results in decreased LAP score |
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Term
|
Definition
-present in up to 95% of CML cases
-caused by exchange of chromosome material (between chromosomes 9 and 22: a new chromosome is formed)
**important in the pathogenesis of CML** |
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Term
|
Definition
| Traditionally the only cure has been bone marrow transplant because CML evolves into acute leukemia and patient dies: median survival has been 3-4 years |
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Term
|
Definition
-"leukemia-like", but benign
-similar CBC findings as CML
-WBC count is > 50,000/uL with predominance of neutrophils and bands; other neutrophil precursor forms are present, but no blasts are present
-is a reactive process
-LAP score is increased because there is a very high absolute neutrophil count with Leukemoid Reaction
-BUT Philadelphia chromosome is absent |
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Term
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
-Incidence- |
|
Definition
1. Most common adult leukemia in US and Western Europe
2. Typically involves elderly patients |
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Term
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
-Symptons- |
|
Definition
1.Fatigue; anorexia (decreased appetite)
2.May be asymptomatic and discovered on routine CBC |
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Term
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
-Physical signs- |
|
Definition
| -Lymphadenopathy (often why patients present);splenomegaly |
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Term
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
-Labs- |
|
Definition
-CBC shows significant lymphocytosis
-(absolute lymphocyte count usually > 10,000/uL)
**If the patient is elderly, worry about CLL**
**LABS- "smudge cells" on peripheral blood smear**
-Neoplastic lymphocytes are delicate and appear smudged
-Bone marrow biopsy:replacement of areas of bone marrow by mature appearing lymphocytes
-Flow cytometry: Can be performed on blood in patients with CLL (or bone marrow biopsy); special surface markers on tumor cells are identified |
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Term
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
-DDX- |
|
Definition
| Differential diagnosis of conditions associated with this very high elevation of the absolute lymphocyte count:Infectious mononucleosis; acute infectious lymphocytosis; pertussis ( "whooping cough" ) |
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Term
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
-Prognosis- |
|
Definition
Typically prolonged survival for many years;
cure at this time is unlikely |
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Term
| BENIGN LYMPH NODE DISORDERS |
|
Definition
ACUTE LYMPHADENITIS
CHRONIC LYMPHADENITIS |
|
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Term
|
Definition
-Acute infection of a lymph node draining an infected site
-Lymph node may develop a suppurative (purulent) infection if primary infection is caused by pyogenic bacteria
-Example: infections of tonsils; teeth drainage of bacteria to lymph nodes in neck (e.g. tonsillar) painful enlargement of lymph node; when severe, abscess may develop (overlying skin may appear erythematous and the site may be swollen) |
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Term
|
Definition
-Chronic enlargement of lymph nodes;can be confused with lymphomas (malignant proliferations of lymphocytes)
-Examples:
1.Bacterial: tuberculosis, cat scratch disease
2.Viral: infectious mononucleosis, HIV
3.Protozoan: toxoplasmosis |
|
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Term
|
Definition
Malignant proliferations of lymphocytes usually presenting as painless enlargement of lymph nodes (lymphadenopathy)
Lymph nodes also firm on physical exam |
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Term
|
Definition
A. Hodgkin's Disease
B. Non-Hodgkin's lymphomas |
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Term
HODGKIN'S DISEASE
-Incidence- |
|
Definition
-2 peaks: young adults; other peak in adults > 50yrs
-Male predominance
-One of the most common cancers in young adults |
|
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Term
HODGKIN'S DISEASE
-Presentation- |
|
Definition
-Tends to present as localized lymphadenopathy
-Many cases asymptomatic
-25-30% have "B symptoms":fever; night sweats; weight loss
- **Reed Sternberg cells on peripheral smear** |
|
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Term
HODGKIN'S DISEASE
-Prognosis- |
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Definition
| Excellent: cure rate = 90% |
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Term
NON-HODGKIN'S LYMPHOMA (N-H-L)
-Incidence & Presentation- |
|
Definition
-all ages
-Localized or generalized lymphadenopathy
-May originate at extranodal site
(e.g. spleen; liver; bone marrow; GI tract; tonsils; brain) |
|
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Term
NON-HODGKIN'S LYMPHOMA (N-H-L)
-Prognosis- |
|
Definition
| Lymphomas may demonstrate high grade behavior (rapid growth; invasiveness of tissue), intermediate grade behavior, and low grade behavior (slow growth) |
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Term
NON-HODGKIN'S LYMPHOMA (N-H-L)
w/ high grade fever |
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Definition
| some very responsive to chemotherapy with good prognosis (high cure rate); others cause death within a few years |
|
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Term
NON-HODGKIN'S LYMPHOMA (N-H-L)
w/ low grade fever |
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Definition
| patients generally survive several years, but cure traditionally has not been possible(New trials with monoclonal antibodies directed against specific B cell receptors are going on which may improve prognosis). |
|
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Term
|
Definition
A.physical exam: palpation of lymph nodes, spleen, liver
B.lymph node biopsy
C.radiologic imaging of chest, abdomen, pelvis
D.CBC and peripheral blood smear
E.bone marrow biopsy
F. flow cytometry(on tissue from lymph nodes, bone marrow etc.)Detects surface markers on cancer cells to identify the cells
G. cytogenetic study (on tissue from lymph nodes, bone marrow etc.) assesses chromosomal abnormalities (e.g. from exchange of material) |
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Term
|
Definition
Malignant proliferation of plasma cells which all secrete the same antibody (monoclonal antibody):
monoclonal antibody is referred to as a paraprotein
-paraproteins may be detected in the blood and/or urine
-involvement of bone marrow by MM plasma cells
-INCIDENCE: older adults |
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Term
MULTIPLE MYELOMA (MM)
-CLINICAL FEATURES- |
|
Definition
-Punched out (destructive or lytic) bone lesions
-caused by accumulations of MM plasma cells in bone marrow;(bone destruction related to cytokine release by plasma cells; diffuse bone destruction may also occur)
-Bone destruction results in hypercalcemia and bone pain (bone pain is most common presenting symptom)
-Bone marrow biopsy: significant # of MM plasma cells in bone marrow |
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Term
MULTIPLE MYELOMA (MM)
-CLINICAL FEATURES-
Serum and/or urine paraprotein |
|
Definition
-antibody made by plasma cells
-Diagnosed by serum and urine protein electrophoresis
-Monoclonal, NOT MULTICLONAL, (M-component) band on ELPH
-Some patients have immunoglobulin light chains that are found by urine protein electrophoresis (Bence Jones proteins) |
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Term
MULTIPLE MYELOMA (MM)
CLINICAL FEATURES cont. |
|
Definition
-Anemia may also be present
-Hypercalcemia
-Renal insufficiency
-Plasmacytomas:skeletal or extraskeletal tumors of malignant plasma cells
-Infections: single paraprotein antibody is made at the expense of the immunoglobulins needed to fight infection |
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Term
HEMATOLOGIC
CAUSES OF SPLENOMEGALY |
|
Definition
-Lymphomas: Hodgkin's disease;non-Hodgkin's lymphomas
-Chronic leukemias:1. chronic myelogenous leukemia (CML)2. chronic lymphocytic leukemia (CLL)
-Acute leukemias
-Polycythemia vera: neoplastic proliferation of RBC in bone marrow (to be discussed in clinical med surg)
-Certain hemolytic anemias (e.g. thalassemia major) |
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Term
| OTHER CAUSES OF SPLENOMEGALY |
|
Definition
Congestive states-1.portal hypertension secondary to chronic liver failure (cirrhosis)
2.right-sided heart failure
-Infectious conditions: e.g. infectious mononucleosis;malaria
-Storage diseases: Enzyme deficiencies result in accumulation of lipids and carbohydrates in spleen and other organs: e.g. Gaucher's disease; Niemann-Pick disease
-Connective tissue disorders: systemic lupus erythematosus; Felty’s syndrome(splenomegaly,lymphadenopathy, neutropenia and rheumatoid arthritis) |
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Term
|
Definition
| Occurs in response to both normal or physiologic conditions (pregnancy), and adverse or pathologic (HTN) conditions |
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Term
|
Definition
| replacement of one differentiated cell type with another, sometimes less differentiated cell type. |
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Term
|
Definition
| refers to abnormal changes in size, shape & organization of mature cells. Often called atypical hyperplasia |
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Term
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Definition
| occurs as a consequence of ischemia or toxic injury |
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Term
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Definition
| if cells are no longer needed, they activate a cellular pathway resulting in cell suicide. Is a continual event |
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Term
|
Definition
Injury may be reversible(sublethal) or irreversible(lethal)
hypoxic
chemical
immunologic
infection
physical agents |
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Term
|
Definition
| lack of O2 & results in “power failure” within the cell. Often due to ischemia. |
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Term
|
Definition
| interruption of blood flow to an area. Most common cause of cell injury. |
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Term
|
Definition
begins with an interaction between a toxic substance & the cells membrane, which is damaged. Increased membrane permeability results in cell damage or death.
ex: carbon tetrachloride,heavy metals (lead, mercury),ethanol |
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Term
| Infectious, immunological cell injury |
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Definition
| cell membranes are injured by direct contact with cellular and chemical components of the immune & inflammatory responses. These include phagocytic cells, histamine, antibodies and proteases. Complement is responsible for many membrane alterations that occur during immunologival injury. |
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Term
|
Definition
intentional or unintentional injuries account for a large portion of cell injury and damage.
ex: temperature extremes, trauma/mechanical stresses, ionizing radiation, atmospheric pressure |
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Term
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Definition
| accompanies ll types of cellular & tissue injury. Includes increased vascular permeability, recruitement & migration of leukocytes and phagocytosis of antigens and debris |
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Term
|
Definition
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Term
|
Definition
antibody formation
Produce active or passive immunity |
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Term
|
Definition
| produced by an individual after exposure (whether produced by the individual or given directly to them) |
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Term
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Definition
| preformed antibodies or T cells are transferred from donor to recipient. Does not involve the host’s immune response at all. |
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Term
|
Definition
– a disturbance in the immunologic tolerance of self-antigens.
ex – lupus erythematosis |
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Term
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Definition
– when the immune system of one person produces an immunologic reaction against tissues of another person.
ex- transfusion rxn |
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Term
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Definition
| when an individual has been immunologically primed, further contact with that antigen leads to secondary boosting of the immune response. an altered immune response to that antigen, resulting in damage to the individual. |
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Term
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Definition
| refers to hypersensitivity to environmental allergens |
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Term
|
Definition
(atopic, anaplylactic): mediated by IgE. Peaks in 15-30min. From T cell deficiency, abnormal mediator feedback, environment
ex – asthma rhinitis atopic eczema
bee sting reaction |
|
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Term
|
Definition
(cytotoxic, cytolytic): mediated IgM or IgG. Peaks in 15-30min. From antigen exposure, foreign cell/tissue/graft.
ex – ABO transfusions, hemolytic disease of
newborn, myasthenia gravis |
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Term
| Type III Hypersensitivity |
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Definition
(immune complex): mediated by IgG. Peaks in 6hrs. From a persistent microbe antigen, self antigen or environmental antigen.
ex – glomerulonephritis, SLE, arthritis |
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Term
|
Definition
(delayed hypersensitivity): mediated by T lymphocytes. Peaks 24-48 hrs. Caused by intradermal, epidermal & dermal antigens.
ex – TB test, contact dermatitis, MS |
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Term
|
Definition
first came to light in 1981 after the unusual occurrence of Pneumocystis Carinii and Kaposi Sarcoma in young men
comprises a set of defined clinical conditions, that are the end result of long-standing HIV infection (10-12 yrs)
is found in every country in the world |
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Term
|
Definition
| HIV-1 was discovered as cause of disease |
|
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Term
|
Definition
cytopathic human retrovirus(destroys)
retrovirus of the lentiviridae family, the virus is single stranded RNA, able to convert their RNA to DNA after they enter the host cell |
|
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Term
|
Definition
convert RNA to DNA after they enter host cell
Means genetic material is in the form of RNA instead of DNA
Reverse transcriptase is the enzyme that transcribes viral RNA into DNA after virus enters target cell
Integrase/protease accompany RNA |
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Term
|
Definition
HIV-1: Predominates in eastern Africa & the rest of the world.
HIV-2: Common in western Africa (less pathogenic). |
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Term
| Mature HIV virion is composed of |
|
Definition
Two molecules of viral RNA in the middle.
(3) enzymes: reverse transcriptase, integrase & protease.
Nucleocapsid composed of 24 proteins:
(2) Glycoproteins, gp 120 and gp 41 are embedded in the lipid bilayer (p17). |
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Term
| Pathogenesity of the Disease |
|
Definition
1.Viral attachment.
2.Fusion & entry into cell.
3.Uncoating of the nucleocapsid & reverse transcription of RNA to copy DNA.
4.DNA migrates into cell nucleus.
5.Integrates into host DNA & initiates transcription to mRNA
6.Viral mRNA translated into viral enzymes & structural proteins.
7.Virion genome (RNA) released into the cytoplasm.
8.Viral RNA combines with core proteins & assembled
9.New HIV virion released from infected CD4+ lymphocyte |
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Term
|
Definition
|
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Term
|
Definition
| (outer coat), lipid bilayer, w/glycoprotein spikes (gp120, gp41) |
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Term
|
Definition
|
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Term
|
Definition
| stem/transmembrane protein |
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Term
|
Definition
| contains the RNA strands and molecules of reverse transcriptase, integrase and protease |
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Term
|
Definition
HIV binds to surface of target cell that has a CD4 receptor
The CD4+ T-Helper lymphocyte is the preferred “target” |
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Term
| cells suspectable to HIV infection |
|
Definition
Monocytes
Macrophages
NK Cells
B Lymphcytes
Endothelial Cells
Langerhans Cells
other various body tissues |
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Term
| What is a Normal Cd4+ Count |
|
Definition
In an intact immune system the CD4+ count ranges from 600-1200/mm3 of blood
A CD4+ count of < 200 (whether symptomatic or asymptomatic) is diagnostic of AIDS*** |
|
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Term
|
Definition
| The median time from HIV infection to progression to AIDs |
|
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Term
| Early symptons of HIV infection |
|
Definition
| Fever, headache, tiredness and enlarged lymph nodes. |
|
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Term
| Late symptons of HIV infection |
|
Definition
| Lack of energy, weight loss, night sweats, persistent yeast infections (oral or vaginal), persistent skin rash and short term memory loss. |
|
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Term
| Symptoms of opportunistic infections in people with AIDS |
|
Definition
Coughing & SOB
Seizures & lack of coordination
Difficult or painful swallowing
Mental symptoms (confusion & forgetfulness)
Severe & persistent diarrhea
Fever
Vision loss
Nausea, abdominal cramps & vomiting
Wt. loss & extreme fatigue
Severe headache
Coma |
|
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Term
| Neuroogical signs of AIDS |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
Pneumocystitis pneumonia
TB
Tumor |
|
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Term
|
Definition
|
|
Term
| signs of AIDS in GI tract |
|
Definition
Esophagitis
Chronic diarrhea
Tumors |
|
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Term
|
Definition
Kaposi sarcoma
Lymphoma
Invasive cervical cancer
Pneumocystis carinii
Herpes Zoster
Mycobacterium avium-intracellulare
Candidiasis
Toxoplasmosis
Cytomegalovirus
Many other opportunistic infections |
|
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Term
| Where is the AIDS?HIV Virus Found |
|
Definition
Blood
Cerebrospinal fluid
Semen & Vaginal secretions
Tears
Urine
Breast milk
Saliva |
|
|
Term
| Three major means of HIV/AIDS transmission are |
|
Definition
| blood, sexual and maternal-infant contact |
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Term
|
Definition
HIV antibodies are NOT at noticeable level in the blood until 3 mo after the infection.
Tests used to detect HIV (AB’s) are:
Enzyme-linked immunosorbent assey (ELISA) twice.
Western blot is performed.
Polymerase chain reaction (PCR). |
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Term
|
Definition
To detect antibodies
1. 2 ELISA tests followed by a confirmatory Western blot confirm with a sensitivity
>95%
2. Most antibodies develop within 6 mos of exposure |
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