Disorders of blood part 2 Flashcards
Lymphoma
Malignant neoplasm involving proliferation of lymphocytes in lymphoid tissue.
All lymphomas are malignant with 35 subtypes.
Lymphoid tissue: thymus, bone marrow, lymph nodes, spleen, tonsils, intestinal lymphoid tissue
Non-hodgkin Lymphoma
Involves T and B cells, originate in extranodal tissue and migrates to contiguous lymph nodes.
Lymph node enlargement and cancerous transformation occur
Non-hodgkin lymphoma etiology
Unknown, but believed to be related to immune system impairment and certain infections
Site specific B and T cells at different levels of differentation migrate to specific parts of the lymph node for which they are normally associated
Non-hodgkin lymphoma classification
B cell or T cell either can be precursor or mature
Non-hodgkin B cell
Mature B cell lymphomas are most common, usually metastasize and involve marrow, impaired humoral immunity is seen with increased susceptibility to bacterial infections
Non-hodgkin clinical manifestations
Lymph node enlargement
Painless gradual lymph swelling
Extranodal sites are nasopharynx, GI tract, bone, thyroid, testes, and soft tissue
Non-hodgkin S&S
Fatigue, malaise, fever, weight loss, pruritis, sweating.
Hypermetabolism, anemia, infections
Approx 15 years survival
Hodgkin Lymphoma
HL features abnormal cells called Reed-Sternberg cells
Distinctive chromosomal abnormalities
Usually B cells are affected, lymphocyte count is decreased, arise in single and and spreads, four distinite types, believed to be related to immune and infections
Hodgkins Lymphoma S&S
Large painless bumps in neck axilla retroperitoneum and inguinal nodes
Fever, weakness, malaise, weight loss, anemia, back and neck pain, pericardial involvement
Can cause pressure or obstruction to extremities and can be painful, nerve irritated or pulseless
Stage of Hodgkins disease
I. One lymph node
II. Two or more lymph nodes same region on same side of diaphragm
III. Lymph nodes on both sides of diaphragm and spleen
IV. Multiple, diffuse
Leukemia
Neoplasms of hematopoietic precursor cells. Caused by gene disruption, unknown etiology but commonly related to treatment of cancer (HL) and EMFs
Common Features of Leukemias
Bone Marrow is infiltrated with malignant cells which replace precursors of erythrocytes, WBC and platelets.
Peripheral blood contains increased number of immature blood cells
Complications of leukemias
Anemia, recurrent infections, uncontrollable bleeding, overwhelming infection commonly causes death
Classification of leukemias
Acute or chronic Myeloid -granulocytic-monocytic Lymphoid - lymphocytic AML ALL CML CLL
ALL or AML
Acute lymphoid or myeloid leukemia
Young people
Proliferation of lymphoblasts (usually B lymphoblasts)
Involves proliferation of precursors for RBC, granulocytes and platelets
Disrupt normal bone function
ALL clinical manifestations
Increased number of immature and nonfunctional lymphocytes in bone marrow cause bone pain and tenderness, infections, decreased RBCs, anemia, poor healing, decreased platelets
Fever, diaphoresis, weight loss, lymphadenopathy, splenomegaly, hepatomegaly, increased viscosity
CLL (most common) or CML
Neoplasms of hematopoietic precursors are more differentiated than in acute, longer survival when untreated
Older people, similar but less severe S&S (night sweats)
Multiple Myeloma
Neoplasm of B cells that mature plasma cells
Multiple malignant tumors of plasma cells scattered throughout excess of IgG and also characterized by multiple bone lesions
Etiology is unknown but linked to immune disorders and infections
Clinical manifestations of multiple myeloma
Bone resoprtion, pain, hypercalcemia, (low phosphate) 66% vertebrae for bone destruction
Low immunoglobins, recurrent infections, related to marrow obstruction (anemia neutropenia and thrombocytopenia)
Hypercoagulability states
Increased platelet function (arterial distrubance or thrombocytosis) or increased activation of coagulation system
Increased platelet function
Endothelial damage or increased platelet sensitivity to factors causing adhesion/aggregations
Increased platelet function
Endothelial damage or increased platelet sensitivity to factors causing adhesion/aggregations
Thrombocytosis is >1000X10^9/L
Primary and secondary (genetic and acquired)
Primary Thrombocytosis
Genetic disorder of thrombopoietic stem cells
Leads to proliferation of potentially non-functional platelets
Increased activity of coagulation system
Primary and secondary causes
Increase in procoagulation factors OR decrease in anticoagulation factors
Primary coagulation
Mutations in genes creates altered clotting factor V which cannot be inactived by protein C (loss of anticoagulation mechanism)
Leads to DVT and can be related to thromboembolism formation in pregnancy
Secondary coagulation issues
Immobility (blood stasis)
Increased viscosity and deformed RBC can also cause lead to stasis via resistance to flow
Paraneoplastic syndrome (certain cancers, troussea syndrome)
Increased Estrogen
Increase in synthesis of coagulation factors by liver and or decrease in synthesis of antithrombin III
Autophospholipid Syndrome-IgG attacks protein-binding phospholipids to intereference with clotting cascad
Bleeding disorders
Thrombocytopenia
Lack of coagulation factors
DIC
Reduced vessel integrity
Thrombcytopenia
< 1000X10^9/L
Decreased production from aplastic anemia, multiple myeloma, radiation, cheo
Increased sequestration from splenomegaly
Increased destruction/use - autoimmune (transfusions, pregnancy) drug induced, DIC
Decreased function from NSAIDS, Von Willebrand Disease
Lack of coagulation factors diseases
Hemophilia
Von Willebrand Disease
Secondary Etiologies
Hemophilia
Sex-linked congenital with forms A and B
A is classic and a deficiency in factor VIII
B (Christmas disease) is a deficiency in IX, transmitted as A
Hemophilia clinical manifestations
Severe is associated with spontaneous bleeding
At 1-5% of normal, bleeding usually after trauma
May experience spontaenous hematuria and epistaxis
PTT and PT used to asses phases
May require transfusions
PT prothrombin time and International normalized ratio (INR)
Measures integrity of extrinsic clotting system as well as factors common to both systems
Also used to monitor effectiveness of coumadin
PTT partial thromboplastin time
Measures integerity of clotting intrinsic system and common components
Often used to monitor standard (unfractionated) heparin anticoagulant therapy
Von Willebrand Disease
vWF platelet adhesion molecule found in platelet granules and endothelium, vWF also carries clotting factor VIII
Secondary Etiologies
Liver disease and Vit K deficiencies
Vit K needed for clotting factors 2,7,9 and 10
DIC
Widespread coagulation and bleeding within the vasculature
Endothelial damage factor XII activation
