Hematological systems disorders Flashcards
1
Q
hematopoiesis
A
- Pluripotential stem cell makes lymphoid progenitor cell and multipotential progenitor cell
- they become committed progenitor cells then maturing cells
- lymphoid progenitor cell becomes lymphocyte
- multipotential progenitor cell becomes everything else
2
Q
Hemochromatosis
A
- over absorption of iron in small intestine
- autosomal recessive hereditary disorder
- 1/250 Northern European
3
Q
Hemochromatosis: pathogenesis
A
- mutations in the genes of any proteins that regulate entry of iron
4
Q
Hemochromatosis: clinical manifestations
A
- bronze skin
- weakness/fatigue
- myalgia/ joint pain
- abdominal pain/constipation
- elevated hemoglobin
- elevated liver enzymes
- Hepatomegaly (enlargement of the liver)
5
Q
Hemochromatosis: treatment
A
- twice weekly phlebotomy where they remove the iron and pump blood back in
6
Q
Anemia
A
- low RBCs
- most commn hematologic abnormality
- HBG <14 for men and less than 12 for women
7
Q
Anemia: causes
A
- excessive blood loss
- acute: trauma, surgery
- chronic: heavy menstruation, GI, or urinary cause
- Destruction of erythrocytes (hemolysis)
- Congential = sickle cell, thalassemia
- acquired
- Decrease production of erythrocytes
- lack of erythropoietin or inability of bone marrow to respond to it
- anemia of chronic disease
- aplastic anemia (bone marrow suppression)
- pernicious anemia Vit B12 deficiency
8
Q
Aplastic Anemia
- what is it and how is it treated
A
- rare, serious
- any age
- body stop producing blood cells
- treatment includes meds, transfusions or bone marrow transplant
9
Q
Anemia of Chronic Disease
A
- occurs in presence of inflammation, infection, and some malignancies
- HBG 7-12 g/dL
- decreased responsiveness to erythropoietin
10
Q
Anemia: clinical manifestations
A
- vary with severity
- fatigue
- weakness
- dyspnea with exertion
- tachycardia
- pallor
- irritability
- headaches
- neuropsychiatric complications
11
Q
Anemia treament
A
- aimed at treating the underlying cause and relieving symptoms
12
Q
Sickle cells anemia
A
- Autosomal recessive
- 1 point mutation that causes changes to RBC
- protective against malaria
13
Q
Sickle cell anemia consequences
A
- jaudice
- infarctions (spleen, kidney, bone, eyes, heart, liver, pulmonary, cerebral)
14
Q
SCA clinical manifestations
A
try to look over dont try to memorize all
- retinal detachment
- retinopathy
- reduced pO2 levels
- pneumonia
- atelectasis
- chest syndrome
- acute hepatomegaly
- gallstones
- hypothenuria
- renal papillary necrosis
- vaso-occlusion
- stasis ulcers in hands ankles and feet
- osteomyelitis
- infections
- splenomegaly
15
Q
Thalassemia
A
- inherited hemoglobinopathies (alpha or beta)
Mediterranean descent - not enough hemoglobin produced
- effects the RBC (membrane, hydration, deformity, oxidation)
- low HBG
- normal RBC
- Fe overload
16
Q
Granulocytes and what they respond to
A
- basophils: allergic reactions
- eosinophils: allergic reactions, parasitic
- Neutrophils: aka polymorphoneuclear leukocyte - first line defense against pathogens
17
Q
Monocytes
A
- largest circulating blood cell
- macrophages once activated
18
Q
Lymphocyte
A
- B & T cells
- antibody production
19
Q
Disorders of leukocytes
A
- leukocytosis: too many
- Leukopenia: too little
- Basophilia: too many basophils
- eosinophilia: too many
- neutrophilia: too many
- Neutropenia: too little
- lymphoctyosis/lymphocytopenia
- monocytosis: too many monocytes (exposure to TB/endocarditis)
20
Q
Acute lymphoblastic leukmia
A
- ALL
- involves the lymophcytes, lymph organs,
- most common in children
- results in immature, functionless cells called blast cells in bone marrow and block development of normal cell development
21
Q
Acute myeloid leukmia
A
- most prevalent acute leukemia and occurs most commonly in adults
- hematopoietic stem cells that are commited to differentiate into myeloid cells (monocytes, granulocytes, erythrocytes and platelets)
- immature cells
- most common over all
22
Q
Chronic lymphocytic leukemia
A
- CLL
- chronic
- overproduction of b lymphs that are mature but not functional
23
Q
chronic myelogenous leukemia
A
- CML
- overproduction of myelogenous cells that are mature but not funcitonal
24
Q
General signs and symptoms of leukemia
A
- headaches
- nausea
- enlarged lymphnodes
- splenomegaly
- decreased production of erythrocytes/anemia
- platelets decrease
- acute is more emergent
25
Q
Types of malignant lymphomas
A
- hodgkin lymphoma
- non hodgkin lymphoma
- multiple myeloma
26
Q
Hodgkin lymphoma
- what is it
- incidence
- treatment
A
- one of the most curable forms of cancer
- lymphoid tissue neoplasms (primarily lymph nodes)
- reed-stern berg cell (b-cell lineage)
- incidence peaks between 25-30 and after 55
- tx = chemo and radiation
27
Q
non-hodgkin lymphoma
A
- Symptoms don’t typically start until later on
- B and T cell malignancies that present as solid tumors
- presentation varies - commonly enlarged chain of lymph nodes
- prognosis depends
- majority older than 55
28
Q
Multiple myeloma
A
- malignant neoplasm of plasma cells starting in bone marrow
- patient at risk for fx
- gradual and insidious onset
- causes anemia, thrombocytopenia and leukopenia
- older adults
- women more than men
- can be secondary cause of something else such RA medications or other medications
29
Q
Signs and symptoms of Hodgkin lymphoma
A
- Severe pruritus
- irregular fever
- jaundice
- hepatosplenomegaly
- superior vena cava syndrome from thrombosis
- pulmonary symptoms (non productive cough, stridor, dyspnea, chest pain, cyanosis and pleural effusion)
- Alcohol-induced bone pain in involved lymph nodes
- invasion of pericardium
- bone pain in back and hips
- Paraplegia
- nerve pain
30
Q
Myeloproligerative neoplasms
A
- excessive cell production in bone marrow
- chronic myelogenous leukemia
- polycythemia vera
- essential thrombocythemia
- primary myelofibrosis
31
Q
polycythemia vera
what is it and what are the phases
A
- increase production in RBC that end up over shadowing the production of normal RBC
- 3 phases:
~ latent
~ proliferative: anemic signs
~ spent: become fibrotic tissue and cause changes in liver and spleen
32
Q
Essential thrombocythemia
A
- platelets >600,00-
- increase production in platelets
- clotting initially and then become at risk for bleeding
- can have primary and secondary causes
- secondary causes such as bleeding disorder and the body over compensates
33
Q
Primary myelofibrosis
A
- CT disorder
- fibrotic tissue in bone marrow
34
Q
Disorders of hemostasis
A
- Von willebrand disease
- hemophilia
- thrombocytopenia
- effects of aspirin and NSAIDs
- disseminated intravascular coagulation
35
Q
Von Willebrand disease
A
- clotting factor dysfunction
- most common inherited bleeding disorder
- defective Von wllebrand factor on chromosome 12
36
Q
hemophilia
A
- bleeding disorder
- sex linked autosomal recessive bleeding disorder
37
Q
- Thrombocytopenia
- effects of aspirin and NSAIDs
A
- low platelets
- can be due to a variety of things and causes fatigue
- Aspring/NSAIDs
- single dose suppresses platelet aggregation for >48 hours
38
Q
Disseminated intravascular coagulation
A
- all over intravascular clotting
- over active coagulation cascade
- cause of simultaneous thrombotic and hemorrhagic event
39
Q
reactions of blood transfusions
A
- febrile non hemolytic reaction
- transfusion-related acute lung injury
- acute hemolytic transfusion reaction
- delayed hemolytic transfusion reaction
- allergic reaction
- anaphylaxis
- septic reaction
40
Q
Implications of hematological system disorder for PT
A
- common signs and symptoms
- precautions with patient
- food/nutrients for anemia
- hematopoietic growth factors: EPO stimulating factors, myeloid growth factors, magakaryocyte growth factors
41
Q
Drugs that facilitate clotting
A
- replacement factors
- vitamin K
- antiplasmin drugs
41
Q
Anticoagulant drugs
A
- anticoagulants
- thromboltics
- anti platelet drugs
42
Q
Anticoagulant drugs
A
- heparin (PTT)
- dabigatran (direct thrombin inhibitors)
- warfarin
- factor Xa inhibitors
43
Q
Thrombolytic drugs
A
- streptokinase
- t-PA derivatives
44
Q
common signs and symptoms of hematologic disorders
A
- edema and congestion
- infarction
- thrombosis and embolism
- lymphedema
- bleeding and bruising
- hypotension and shock
44
Q
Antiplatelet druges
A
- Aspirin
- ADP receptor inhibitors
- glycoprotein lab/lla inhibitors
- PAR-1 receptor inhibitors
- PDE3/Adenosine uptake inhibitors
45
Q
Clinical Signs and symptoms of hemophilia bleeding episodes: GI involvement
A
- abdominal pain and distention
- melena (blood in stool)
- hematemesis (vomiting blood)
- fever
- low abdominal/groin pain from bleeding into wall of large intestine or iiliopsoas muscle
- hip flexion contraction due to spasm of the iliopsoas muscle secondary to retroperitoneal hemorrhage
46
Q
Clinical Signs and symptoms of hemophilia bleeding episodes: CNS involvement
A
- impaired judgment
- decreased visual and spatial awareness
- short term memory deficits
- inappropriate behavior
- motor deficits: spasticity, ataxia, abnormal gait, apraxia, decreased balance, loos of coordination
47
Q
Supervised exercise program for people with bleeding disorders and hemophilic arthritis
A
- progression to next level only if no adverse reaction
- prophylaxis: factor infusion recommended for people with sever hemophilia; people with mild and moderate hemophilia to have medications available if needed
- intensity: precent of isometric Nicholas dynamometry muscle test to assess pounds of weight to use or color of theraband
- Repetitions: to be done only in pain-free range
- Rate: 5-10 seconds concentric with exhale; 5-10 seconds eccentric with inhale
48
Q
Clinical signs and symptoms of acute hemarthrosis
A
- tingling sensation
- stiffening into the position of comfort
- decrease ROM
- pain/tenderness
- swelling
- protective muscle spasms
- increase warmth around a joint
49
Q
Clinical signs and symptoms of muscle hemorrhage
A
- gradual intensifying pain
- protective spasm of muscle
- limitation of movement at the surrounding joints
- muscle assumes a position of comfort
- loss of sensation
