Disorders of blood part 2

Question 1

Lymphoma

Answer 1

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

Question 2

Non-hodgkin Lymphoma

Answer 2

Involves T and B cells, originate in extranodal tissue and migrates to contiguous lymph nodes.
Lymph node enlargement and cancerous transformation occur

Question 3

Non-hodgkin lymphoma etiology

Answer 3

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

Question 4

Non-hodgkin lymphoma classification

Answer 4

B cell or T cell either can be precursor or mature

Question 5

Non-hodgkin B cell

Answer 5

Mature B cell lymphomas are most common, usually metastasize and involve marrow, impaired humoral immunity is seen with increased susceptibility to bacterial infections

Question 6

Non-hodgkin clinical manifestations

Answer 6

Lymph node enlargement
Painless gradual lymph swelling
Extranodal sites are nasopharynx, GI tract, bone, thyroid, testes, and soft tissue

Question 7

Non-hodgkin S&S

Answer 7

Fatigue, malaise, fever, weight loss, pruritis, sweating.
Hypermetabolism, anemia, infections
Approx 15 years survival

Question 8

Hodgkin Lymphoma

Answer 8

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

Question 9

Hodgkins Lymphoma S&S

Answer 9

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

Question 10

Stage of Hodgkins disease

Answer 10

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

Question 11

Leukemia

Answer 11

Neoplasms of hematopoietic precursor cells. Caused by gene disruption, unknown etiology but commonly related to treatment of cancer (HL) and EMFs

Question 12

Common Features of Leukemias

Answer 12

Bone Marrow is infiltrated with malignant cells which replace precursors of erythrocytes, WBC and platelets.
Peripheral blood contains increased number of immature blood cells

Question 13

Complications of leukemias

Answer 13

Anemia, recurrent infections, uncontrollable bleeding, overwhelming infection commonly causes death

Question 14

Classification of leukemias

Answer 14

Acute or chronic
Myeloid -granulocytic-monocytic
Lymphoid - lymphocytic
AML
ALL
CML
CLL

Question 15

ALL or AML

Acute lymphoid or myeloid leukemia

Answer 15

Young people
Proliferation of lymphoblasts (usually B lymphoblasts)
Involves proliferation of precursors for RBC, granulocytes and platelets
Disrupt normal bone function

Question 16

ALL clinical manifestations

Answer 16

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

Question 17

CLL (most common) or CML

Answer 17

Neoplasms of hematopoietic precursors are more differentiated than in acute, longer survival when untreated
Older people, similar but less severe S&S (night sweats)

Question 18

Multiple Myeloma

Answer 18

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

Question 19

Clinical manifestations of multiple myeloma

Answer 19

Bone resoprtion, pain, hypercalcemia, (low phosphate) 66% vertebrae for bone destruction
Low immunoglobins, recurrent infections, related to marrow obstruction (anemia neutropenia and thrombocytopenia)

Question 20

Hypercoagulability states

Answer 20

Increased platelet function (arterial distrubance or thrombocytosis) or increased activation of coagulation system

Question 21

Increased platelet function

Answer 21

Endothelial damage or increased platelet sensitivity to factors causing adhesion/aggregations

Question 22

Increased platelet function

Answer 22

Endothelial damage or increased platelet sensitivity to factors causing adhesion/aggregations
Thrombocytosis is >1000X10^9/L
Primary and secondary (genetic and acquired)

Question 23

Primary Thrombocytosis

Answer 23

Genetic disorder of thrombopoietic stem cells

Leads to proliferation of potentially non-functional platelets

Question 24

Increased activity of coagulation system

Answer 24

Primary and secondary causes

Increase in procoagulation factors OR decrease in anticoagulation factors

Question 25

Primary coagulation

Answer 25

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

Question 26

Secondary coagulation issues

Answer 26

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

Question 27

Bleeding disorders

Answer 27

Thrombocytopenia
Lack of coagulation factors
DIC
Reduced vessel integrity

Question 28

Thrombcytopenia

Answer 28

< 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

Question 29

Lack of coagulation factors diseases

Answer 29

Hemophilia
Von Willebrand Disease
Secondary Etiologies

Question 30

Hemophilia

Answer 30

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

Question 31

Hemophilia clinical manifestations

Answer 31

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

Question 32

PT prothrombin time and International normalized ratio (INR)

Answer 32

Measures integrity of extrinsic clotting system as well as factors common to both systems
Also used to monitor effectiveness of coumadin

Question 33

PTT partial thromboplastin time

Answer 33

Measures integerity of clotting intrinsic system and common components
Often used to monitor standard (unfractionated) heparin anticoagulant therapy

Question 34

Von Willebrand Disease

Answer 34

vWF platelet adhesion molecule found in platelet granules and endothelium, vWF also carries clotting factor VIII

Question 35

Secondary Etiologies

Answer 35

Liver disease and Vit K deficiencies

Vit K needed for clotting factors 2,7,9 and 10

Question 36

DIC

Answer 36

Widespread coagulation and bleeding within the vasculature

Endothelial damage factor XII activation