Alterations of leukocyte, lymphoid, and hemostatic function Flashcards
Leukopenia
Deficiencies
-See w/ radiation, chemo, autoimmune disorders
Leukocytosis
Leukocytes increased
-can occur w/ high dose steroids, infections, inflammation
Quantitative disorders
Decreased production in bone marrow or increase in cell destruction in circulation
Qualtitative disorders
Phagocytes lose ability to function. Lymphocytes loose capacity to respond to antigens (mono, leukemias)
Granulocytosis (Neutrophils, basophils, eosinophils)(Neutropenia)
-Present in first stages of infection or inflammation
Granulocytopenia (agranulocytosis )
- causes: interference c/ hematopoiesis, immune mechanisms, chemotherapy destruction, ionizing radiation.
- Can result in recurrent persistent life threatening infections (particularly in respiratory system)
- Sepsis caused by this can result in death w/in 3-6 days
- S/S: infection (recurrent), sepsis, malaise, fever, tachycardia, mouth sores
Eosinophilia
- Eosinophils are increased
- Hypersenstivity reactions trigger the release (parasites, allergies)
Eosinopenia
- Eosinophil count decreases
- Causes: surgery, shock, trauma, burns, mental distress, cushing syndrome
- Migration of cells to inflammatory sites
Basophilia
- Circulating numbers of basophils increase
- occurs in inflammation and hypersensitivity reactions
- Contains histamine that is released in allergic reactions
Basopenia
- decrease in numbers
- occurs in acute infections, hyperthyroidism and long-term steroid therapy
Monocytosis
- numbers increase
- occurs w/ neutropenia in later stages of bacterial infections
- found in chronic infection and correlates w/ extent of myocardial damage
Monocytopenia
- Numbers decrease
- Prednisone treatments, hairy cell leukemia
Lymphocytosis
- increases
- occurs from acute viral infections (epstein-barr virus)
- Other causes: leukemia, lymphomas, some chronic infections
Lymphocytopenia
- count decreases
- immune deficiencies, drug destruction, viral desctruction, radiation, or AIDS
Mononucleosis
- viral infection of B lymphocytes
- commonly caused by EBV
- Transmission: saliva, GU, rectal, respiratory and blood
Mono
- S/S: Malaise, athraglia, fever, pharyngitis, lymphadenopathy, cervical lymph nodes
- Dx: monospot qualitative test for heterophilic antibodies
- treatment: rest and alleviation of symptoms w/ antipyretics, penicillin or erythromycin, ibuprofen.
Leukemias
- malignant disorders of blood and blood forming organs
- uncontrolled proliferation of malignant leukocytes (overproduction of bone marrow, decreased production & function of normal hematopoietic cells
- seen in myeloid or lymphoid and can be acute or chronic
Myeloid cells
-neutrophils, red cells, platelets
AML-most common in adults
-ALL-kids
Blast cells
-more blast cells in the bone marrow aren’t good
Leukemia risk factors
- cigarette smoking, exposure to benzene, ionizing radiation
- HIV, Hep C, HTLV
- Pancytopenia (reduction in all cellular components of blood)
Acute Lymphocytic Leukemia (ALL)
- Most common in kids
- Genetic anomaly: philadelphia chromosome: translocation between chromosomes 9 and 22
Risk factors of ALL
- Prenatal xray exposure
- postnatal exposure to high-dose radiation
- HTLV
- Down syndrome
Acute Myelogenous Leukemia (AML)
- Most common in adult leukemias (mean age 67)
- Down syndrome increases risk
- Abnormal proliferation of myeloid precursor cells
- Decreased rate of apoptosis (cells aren’t dying off like they should)
- Arrest in cellular differentiation is a result
- Mutations in receptor tyrosine kinase FLT3
- Risks: exposure to radiation, benzene, and chemo, hereditary conditions
AML manifesations
- Fatigue caused by anemia
- bleeding resulting from thrombocytopenia, fever from infection, anorexia, wt loss, diminished sensitivity to sour or sweetness, wasting of muscle and difficulty swallowing, CNS involvment
AML dx and tx
- DX: Bone marrow bx, blood count, LP to check CSF for leukemic cells, peripheral blood smear
- TX: induction chemo, then ALLO HSCT. Supportive measures: blood transfusions, abx, allopurinol, stem cell transplant, antifungals, abx, antivirals
Complications of leukemia
Anemia (give blood products), neutropenia (give granulocyte colony stimulating factor-GCSF) or GM-CSF). Low WBC count
Chronic myelogenous leukemia (CML)
- usually diagnosed in adults
- Myeloproliferative disorder that also includes polycythemia vera, primary thrombocytosis, and idiopathic myelofibrosis
- Philadelphia chromosome is often present in BCR-ABL1 causes initiation of CML
Chronic Lymphocytic leukemia (CLL)
- Affects monoclonal B lymphocytes
- Familial tendency
- Common in adults older than 50
- Asymptomatic at the time of dx, lymphadenopathy is most common finding, suppresses humoral immunity and increases infection w/ encapsulated bacteria
CML s/s
- Infections, fever, wt loss,
- Chronic phase: 2-5 yrs-symptoms may not be apparent
- Accelarated phase: 6-18 months-primary symptoms develop: splenomegaly
- Terminal blast phase: blast crisis: survival is 3-6 months
Tests/Tx for CML and CLL
- Blood smear
- Tx: chlorambucil, adminstered w/ or without corticosteroids, chemo
- no cure for CML: Combined chemo, biologic response modifiers, allogenic stem cell transplant
Leukemia Table
ALL:
-Rapid, most common in kids, 91% survival, greater than 30% lymphoblasts and B cells
CLL
-Slow, common in adults, 85% survival, monoclonal B
AML
-Rapid, common in adult form, 24% survival, precursor to myeloid cells
CML
-Slow, found mostly in adults, no cure, neutrophilic or eosinophilic C or clonal: arise from a hematopoetic stem cell
Lymphadenopathy
-Enlarged lymph nodes that become palpable and tender
Local: drainage of an inflammatory lesion located near the enlarged node
General: Occurs in the presence of malignant or nonmalignant disease
Causes of lymphadenopathy
- Neoplastic disease
- Immunologic or inflammatory conditions
- Endocrine disorders
- Lipid storage diseases
- Unknown causes
Primary lymphoid tissue
-Thymus, bone marrow
Secondary Lymphoid tissue
-Lymph nodes, spleen, tonsils. intestinal lymphoid tissue
Malignant lymphomas (Hodgkin lymphoma, non-hodgkin lymphoma)
- Hodgkin lymphoma: Linked to EBV
- Non-hodgkin lymphoma: result from genetic mutations or a viral infection
Hodgkin lymphoma
- Reed sternberg cells in the lymph nodes
- RS cells are necessary for dx but not specific to hodgkin along w/ symptoms
- Types: classic hodgkin lymphoma, nodular lymphocyte-predominant hodgkin
Clinical manifestations of hodgkin lymphoma
- Enlarged painless neck lymph nodes
- Lymphadenopathy causing pressure or obstruction
- Mediastinal mass
- Fever, weight loss, night sweats, pruritus, fatigue
- Tests: CXR, lymphangiography, and biopsy
- Stage 2 is above and below diaphragm
Treatment of HL
- Cure rate is 75%. combined treatment with radiation therapy, and chemotherapy.
- High dose chemo w/ bone marrow stem cell transplant
- Monoclonal antibodies
- nonmyelobative allogenic stem cell transplant
Non-Hodgkin lymphoma
- now called B cell neoplasms and includes T cell and natural killer (NK) neoplasms
- Linked to chromosome translocations
- Expansions of B cells, T cells, NK cells
- Three phases
- Dx: bone marrow bx, CT CAP, CBC
NHL manifestations
-ascitis, leg swelling, retroperitoneal and abdominal masses, nodal enlargement
Comparison of NHL and HL
HL: -nodes are localized (cervical, mediastinal, para-aortic), -extranodal involvement is rare, -spread is orderly, -fever, night sweats and weight loss is common, -reed-sternberg cells are present, -extent of disease is localized NHL -Multiple peripheral nodes -Extranodal involvement is common -Spread is not contagious -fever, night sweats, weight loss is uncommon -Reed sternberg cells are not present -Extent of disease is rarely localized
Symptoms/conditions that mimic lymphomas
-TB, syphilis, systemic lupus, erythematosus, lung cancer, bone cancer
Plasma cell malignancies (Multiple myeloma)
- plasma cells proliferate in the bone marrow: translocation involves immunoglobin heavy chain on chromosome 14
- s/s: hypercalcemia, renal failure, anemia, lytic lesions, skeletal pain, hyperviscosity syndrome, recurring infections
Tx of mulitple myeloma
Prognosis is poor, combinations of chemotherapy, radiation, high dose chemo followed by blood forming stem cell transplantation, bisphophonates (to reduce skeletal damage), hydration and diuretics, abx
Waldenstrom macroglobulinemia (Lymphoplamacytic lymphoma)
-Rare type of slow growing plasma cell tumor that secretes IgM molecule
Splenomegaly
May be classified as pathologic
-May or may not have disease
Hypersplenism
Overactive spleen
-See leukopenia, TCP, anemia, cellular bone marrow.
Congestive splenomegaly
occurs with hepatic cirrhosis
-Seen with ascites, portal HTN, esophageal varices
Thrombocytopenia
<50,000 hemorrhage from minor trauma
<15,000 spontaneous bleeding
<10,000 severe bleeding that can be fatal
Immune thrombocytopenic purpura (ITP)
- IgG antibody targets platelet glycoproteins
- Antibody-coated platelets are sequestered and removed from circulation
- Acute form develops after viral infections (common childhood bleeding disorder)
- Chronic form usually is found in adults
- S/S: petechiae, purpura, progressing to major hemorrhage
- Treatment: palliative, not curative, prednisone, romiplostim, eltrombopag, splenectomy
Thrombotic Thrombocytopenic purpura (TTP)
-Platelets aggregate and cause occlusion of arterioles and capillaries
Thrombocytosis (Thrombocythemia)
- Platelet >400,000
- Accelerated platelet production in the bone marrow
DIC (Disseminated intravascular coagulation)
- Clotting and hemorrhaging simultaneously
- Causes: sepsis, cancer, acute leukemia, trauma blood transfusion
- Increase in fibrin and thrombin activity in the blood
- Magnitude of clotting may result in increased consumption of plts=bleeding
- Can be caused by sepsis due to endothelial damage
- Give plts, FFP, cryoprecipitate-replaced fibrinogen
