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
Types of malignant lymphomas
- hodgkin lymphoma
- non hodgkin lymphoma
- multiple myeloma
26
Hodgkin lymphoma
- what is it
- incidence
- treatment
- 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
non-hodgkin lymphoma
- 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
Multiple myeloma
- 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
Signs and symptoms of Hodgkin lymphoma
- 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
Myeloproligerative neoplasms
- excessive cell production in bone marrow
- chronic myelogenous leukemia
- polycythemia vera
- essential thrombocythemia
- primary myelofibrosis
31
polycythemia vera
| what is it and what are the phases
- 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
Essential thrombocythemia
- 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
Primary myelofibrosis
- CT disorder
- fibrotic tissue in bone marrow
34
Disorders of hemostasis
- Von willebrand disease
- hemophilia
- thrombocytopenia
- effects of aspirin and NSAIDs
- disseminated intravascular coagulation
35
Von Willebrand disease
- clotting factor dysfunction
- most common inherited bleeding disorder
- defective Von wllebrand factor on chromosome 12
36
hemophilia
- bleeding disorder
- sex linked autosomal recessive bleeding disorder
37
1. Thrombocytopenia
2. effects of aspirin and NSAIDs
1. low platelets
- can be due to a variety of things and causes fatigue
2. Aspring/NSAIDs
- single dose suppresses platelet aggregation for >48 hours
38
Disseminated intravascular coagulation
- all over intravascular clotting
- over active coagulation cascade
- cause of simultaneous thrombotic and hemorrhagic event
39
reactions of blood transfusions
- febrile non hemolytic reaction
- transfusion-related acute lung injury
- acute hemolytic transfusion reaction
- delayed hemolytic transfusion reaction
- allergic reaction
- anaphylaxis
- septic reaction
40
Implications of hematological system disorder for PT
- common signs and symptoms
- precautions with patient
- food/nutrients for anemia
- hematopoietic growth factors: EPO stimulating factors, myeloid growth factors, magakaryocyte growth factors
41
Drugs that facilitate clotting
- replacement factors
- vitamin K
- antiplasmin drugs
41
Anticoagulant drugs
- anticoagulants
- thromboltics
- anti platelet drugs
42
Anticoagulant drugs
- heparin (PTT)
- dabigatran (direct thrombin inhibitors)
- warfarin
- factor Xa inhibitors
43
Thrombolytic drugs
- streptokinase
- t-PA derivatives
44
common signs and symptoms of hematologic disorders
- edema and congestion
- infarction
- thrombosis and embolism
- lymphedema
- bleeding and bruising
- hypotension and shock
44
Antiplatelet druges
- Aspirin
- ADP receptor inhibitors
- glycoprotein lab/lla inhibitors
- PAR-1 receptor inhibitors
- PDE3/Adenosine uptake inhibitors
45
Clinical Signs and symptoms of hemophilia bleeding episodes: GI involvement
- 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
Clinical Signs and symptoms of hemophilia bleeding episodes: CNS involvement
- 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
Supervised exercise program for people with bleeding disorders and hemophilic arthritis
1. progression to next level only if no adverse reaction
2. prophylaxis: factor infusion recommended for people with sever hemophilia; people with mild and moderate hemophilia to have medications available if needed
3. intensity: precent of isometric Nicholas dynamometry muscle test to assess pounds of weight to use or color of theraband
4. Repetitions: to be done only in pain-free range
5. Rate: 5-10 seconds concentric with exhale; 5-10 seconds eccentric with inhale
48
Clinical signs and symptoms of acute hemarthrosis
- tingling sensation
- stiffening into the position of comfort
- decrease ROM
- pain/tenderness
- swelling
- protective muscle spasms
- increase warmth around a joint
49
Clinical signs and symptoms of muscle hemorrhage
- gradual intensifying pain
- protective spasm of muscle
- limitation of movement at the surrounding joints
- muscle assumes a position of comfort
- loss of sensation
