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Medical Conditions 2 > Hematological systems disorders > Flashcards

Hematological systems disorders Flashcards

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USMLE® Step 1 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
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2
Q

Hemochromatosis

A
  • over absorption of iron in small intestine
  • autosomal recessive hereditary disorder
  • 1/250 Northern European
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3
Q

Hemochromatosis: pathogenesis

A
  • mutations in the genes of any proteins that regulate entry of iron
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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)
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5
Q

Hemochromatosis: treatment

A
  • twice weekly phlebotomy where they remove the iron and pump blood back in
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6
Q

Anemia

A
  • low RBCs
  • most commn hematologic abnormality
  • HBG <14 for men and less than 12 for women
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7
Q

Anemia: causes

A
  1. excessive blood loss
  • acute: trauma, surgery
  • chronic: heavy menstruation, GI, or urinary cause
  1. Destruction of erythrocytes (hemolysis)
  • Congential = sickle cell, thalassemia
  • acquired
  1. 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
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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
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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
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10
Q

Anemia: clinical manifestations

A
  • vary with severity
  • fatigue
  • weakness
  • dyspnea with exertion
  • tachycardia
  • pallor
  • irritability
  • headaches
  • neuropsychiatric complications
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11
Q

Anemia treament

A
  • aimed at treating the underlying cause and relieving symptoms
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12
Q

Sickle cells anemia

A
  • Autosomal recessive
  • 1 point mutation that causes changes to RBC
  • protective against malaria
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13
Q

Sickle cell anemia consequences

A
  • jaudice
  • infarctions (spleen, kidney, bone, eyes, heart, liver, pulmonary, cerebral)
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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

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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
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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
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17
Q

Monocytes

A
  • largest circulating blood cell
  • macrophages once activated
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18
Q

Lymphocyte

A
  • B & T cells
  • antibody production
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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)
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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
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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
  1. Thrombocytopenia
  2. effects of aspirin and NSAIDs
A
  1. low platelets
  • can be due to a variety of things and causes fatigue
  1. 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
  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
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

Medical Conditions 2 (22 decks)

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