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Clinical Therapeutics 2 > Week 9 - Haematology > Flashcards

Week 9 - Haematology Flashcards

1
Q

Lymphoid Organs consist of Primary and Secondary

A

Primary: Thymus and bone marrow
Secondary: Spleen, lymph nodes, tonsils, peyers patches

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2
Q

Anaemia

A 
Reduction in the total number of erythrocytes in the circulating blood or in the quality or quantity of hemoglobin due to:
Impaired erythrocyte production
Acute or chronic blood loss
Increased erythrocyte destruction
Combination of the above
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3
Q

Symptoms of Anaemia

A

Fatigue, weakness, dyspnea, and pallor

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4
Q

Physiological manifestation of Anaemia

A

Reduced oxygen carrying capacity

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5
Q

MACROCYTIC-NORMOCHROMIC ANAEMIA

A

Macrocytic-large cell

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6
Q

Pernicious Anaemia

A

Caused by a lack of intrinsic factor required for vitamin B12 absorption
Results in vitamin B12 deficiency

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7
Q

Folate deficiency anaemia

A

Always caused by lack of folate in the diet
Similar symptoms to pernicious anemia
Mechanism of cause unclear
Folate deficiency also causes neural tube defects in the foetus

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8
Q

MICROCYTIC-HYPOCHROMIC ANAEMIA

A

Microcytic-small cell
Hypochromic-reduced levels of haemoglobin
Related to:
Disorders of iron metabolism
Disorders of porphyrin and heme synthesis
Disorders of globin synthesis

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9
Q

Iron deficiency anemia

A

Most common type of anaemia worldwide
Nutritional iron deficiency
Metabolic or functional deficiency
Progression of iron deficiency causes:
Brittle, thin, coarsely ridged, and spoon-shaped nails
A red, sore, and painful tongue
Treatment is by iron replacement therapy (usually ferrous sulfate)

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10
Q

Sideroblastic anemia

A

Altered mitochondrial metabolism causing ineffective iron uptake and resulting in dysfunctional hemoglobin synthesis
Ringed sideroblasts within the bone marrow are diagnostic
Sideroblasts are erythroblasts that contain iron granules that have not been synthesized into hemoglobin
Erythropoietic hemochromatosis or iron overload is usually present

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11
Q

NORMOCYTIC-NORMOCHROMIC ANAEMIA

A

Characterized by red cells that are relatively normal in size and hemoglobin content but insufficient in number

Five distinct groups

  • Aplastic anaemia
  • Posthaemorrhagic anaemia
  • Haemolytic anaemia
  • Sickle cell anaemia
  • Anaemia of chronic inflammation
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12
Q

Aplastic anemia usually means

A

Pancytopenia (reduction or absence of all 3 blood cell types)
Results from failure or suppression of bone marrow
Treatment is with immunotherapy and bone marrow transplant

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13
Q

Posthaemorrhagic anemia

A

Acute blood loss from the vascular space

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14
Q

Hemolytic anaemia is caused by

A

Accelerated destruction of red blood cells

Divided into inherited or acquired conditions
Treated by removing cause of problem, or treating under lying cause

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15
Q

Sickle cell anaemia

A

Includes sickle cell thalassemia and sickle cell-Hb C disease
Inherited autosomal recessive disorder

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16
Q

Anaemia of chronic inflammation

A

Mild to moderate anemia seen in:
AIDS, rheumatoid arthritis, lupus, hepatitis, renal failure, and malignancies
Pathologic mechanisms
Decreased erythrocyte life span
Ineffective bone marrow response to erythropoietin
Altered iron metabolism

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17
Q

POLYCTTHAEMIA

A

Overproduction of red blood cells
Relative polycythemia
Result of dehydration
Fluid loss results in relative increases of red cell counts and Hb and Hct values

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18
Q

Absolute polycythemia

A

Primary absolute

Abnormality of stem cells in the bone marrow

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19
Q

Secondary absolute polycythemia

A

Increase in erythropoietin as a normal response to chronic hypoxia or by erythropoietin-secreting tumors

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20
Q

Leukocytosis

A

Normal protective response to physiologic stressors

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21
Q

Leukopenia

A

A low white count predisposes a patient to infections

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22
Q

Neutrophil

A

↑Acute infection; ↓ prolonged infection

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23
Q

Eosinophil

A

↑Allergy and parasite; ↓surgery, shock, trauma

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24
Q

Basophil

A

↑Inflammation and allergy; ↓ Hyperthyroidism and steroid therapy

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25
Q

Lymphocyte

A

↑ acute viral infection; ↓ drug destruction, viral destruction

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26
Q

INFECTIOUS MONONUCLEOSIS

A

Acute, self-limiting infection of B lymphocytes transmitted by saliva through personal contact

  • Commonly caused by the Epstein-Barr virus (EBV)—85%
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27
Q

Symptoms of infectious mononucleosis

A

Symptoms: fever, sore throat, swollen cervical lymph nodes, increased lymphocyte count, and atypical (activated) lymphocytes

28
Q

Diagnostic Test for infectious mononucleosis

A

Monospot qualitative test for heterophilic antibodies

29
Q

Leukemia

A

Malignant disorder of the blood and blood-forming organs

Excessive accumulation of leukemic cells

30
Q

Acute Leukemia

A

Presence of undifferentiated or immature cells, usually blast cells

31
Q

Chronic Leukaemia

A

Predominant cell is mature but does not function normally

32
Q

4 classifications for leukaemia

A

Lymphocytic (lymph gland)
Myeloid (bone marrow)
Acute (immature cells)
Chronic (mature, dysfunctional cells)

33
Q

Signs and symptoms of leukaemias

A 
Anemia
Bleeding purpurea, petechiae (red spots) and ecchymosis (bruise) 
Thrombosis, hemorrhage and DIC
Infection
Weight loss
Bone pain
Elevated uric acid
Liver, spleen, and lymph node enlargement
34
Q

MYELOMA

A

Proliferation of plasma cells
The tumor may be solitary or multifocal (multiple myeloma)
The malignant plasma cells produce abnormally large amounts of one class of immunoglobulin or incomplete immunoglobulin
The unattached light chains of the immunoglobulins (Bence Jones proteins) can pass through the glomerulus and damage the renal tubular cells

35
Q

Multiple myeloma causes increased osteoclastic bone destruction. Clinical manifestations are:

A

Cortical and medullary bone loss
Skeletal pain
Recurring infections due to loss of the humoral immune response

36
Q

LYMPHADENOPATHY

A

Enlarged lymph nodes that become palpable and tender
Local lymphadenopathy
Drainage of an inflammatory lesion located near the enlarged node
General lymphadenopathy
Occurs in the presence of malignant or nonmalignant disease
Neoplastic disease
Inflammatory conditions
Endocrine disorders
Lipid storage disease

37
Q

MALIGNANT LYMPHOMAS

A

Malignant transformation of a lymphocyte and proliferation of lymphocytes, histiocytes, their precursors, and derivatives in lymphoid tissues

Two major categories
Hodgkin lymphoma
Non-Hodgkin lymphomas

38
Q

HODGKINS LYMPHOMA

A

Characterized by the presence of Reed-Sternberg cells in the lymph nodes
Reed-Sternberg cells are necessary for diagnosis, but not specific to Hodgkin’s

Divided into:

  • Classical Hodgkin lymphoma
  • Nodular lymphocyte predominant Hodgkin lymphoma
39
Q

Non-Hodgkin lymphomas are linked to

A

Chromosome translocations, viral and bacterial infections, environmental agents, immunodeficiencies, and autoimmune disorders

40
Q

Burkitt Lymphoma

A

Most common type of non-Hodgkin lymphoma in children
Burkitt lymphoma is a very fast-growing tumor of the jaw and facial bones
Epstein-Barr virus is found in nasopharyngeal secretions of patients

41
Q

SPLENIC FUNCTION

A

Largest of the secondary lymphoid organs
Serves as blood reservoir
Supplies lymphocytes for response to blood born microorganisms

42
Q

Thrombocytopenia

A 
Platelet count <100,000/mm3
Effects of low platelet count are
<50,000/mm3—hemorrhage from minor trauma
<15,000/mm3—spontaneous bleeding
<10,000/mm3—severe bleeding
43
Q

Causes of Thrombocytopenia

A

Hypersplenism, autoimmune disease, hypothermia, and viral or bacterial infections that cause disseminated intravascular coagulation (DIC)

44
Q

Immune thrombocytopenic purpurea (ITP)

A

Autoimmune disorder where antibodies target platelet glycoprotein
The acute form of ITP that often develops after a viral infection is one of the most common childhood bleeding disorders

45
Q

Clinical manifestations of Immune thrombocytopenic purpurea (ITP)

A

Petechiae and purpurea, progressing to major hemorrhage

46
Q

Thrombotic thrombocytopenic purpurea (TTP)

A

Unknown cause

A thrombotic microangiopathy
Platelets aggregate, form microthrombi, and cause occlusion of arterioles and capillaries
Chronic relapsing TTP-recurring episodes at approximately 3 week intervals
Acute idiopathic TTP-more common. Fatal if left untreated

Treatments include steroids, plasma exchange or plasmapherisis or splenectomy if treatments unsuccessful

47
Q

Essential (primary) thrombocythemia

A

Thrombocythemia is characterized by platelet counts >600,000/mm3
Myeloproliferative disorder of platelet precursor cells
Megakaryocytes in the bone marrow are produced in excess
Microvasculature thrombosis occurs

48
Q

Alterations to platelet function

A

Demonstrate an increased bleeding time in the presence of a normal platelet count
Platelet function disorders result from deficiencies in platelet membrane glycoprotein and von Willebrand factor

Manifestations
Petechiae, purpurea, mucosal bleeding, gingival bleeding, spontaneous bruising
Disorders can be congenital or acquired

49
Q

ALTERATIONS OF COAGULATION

A

Vitamin K deficiency
Vitamin K is necessary for synthesis and regulation of prothrombin, the prothrombin factors (II, VII, XI, X), and proteins C and S (anticoagulants)
Liver disease
Liver disease causes a broad range of homeostasis disorders
Defects in coagulation, fibrinolysis, and platelet number and function

50
Q

DISSEMINATED INTRAVASCULAR COAGULATION (DIC)

A

Complex, acquired disorder in which clotting and hemorrhage occur simultaneously
DIC is the result of increased protease activity in the blood caused by unregulated release of thrombin with subsequent fibrin formation and accelerated fibrinolysis
Endothelial damage is the primary initiator of DIC in the presence of sepsis

51
Q

Clinical signs and treatment of (DIC)

A

Bleeding from venipuncture sites
Bleeding from arterial lines
Purpurea, petechiae, and haematomas
Symmetric cyanosis of the fingers and toes

Treatment is to
Remove the stimulus
Restore haemostasis
Maintain organ viability

52
Q

Iron deficiency anaemia in children

A

Most common blood disorder of infancy and childhood
Lack of iron intake or blood loss

Manifestations
Irritability, decreased activity tolerance, weakness, and lack of interest in play

53
Q

Acquired congenital hemolytic anemia

A

Hemolytic disease of the newborn (HDN)
Alloimmune disease
Maternal antibody directed against fetal antigens
ABO incompatibility occurs in 20% to 25% of cases
Rh incompatibility occurs in less than 10%
Also termed erythroblastosis fetalis

54
Q

Hemolytic disease of the newborn (HDN)

A 
Manifestations:
Anemia
Hyperbilirubinemia
Icterus neonatorum (neonatal jaundice)
Kernicterus (bilirubin deposition in the brain)
55
Q

Anemia of infectious disease

A

Diseases initially acquired by the mother and transmitted to the fetus
Results in hemolytic anemia
Likely due to injury to the erythrocyte membranes or erythrocyte precursors by infectious organism

56
Q

Glucose-6-phosphate dehydrogenase deficiency (G6PD)

A

Inherited, X-linked, recessive disorder
G6PD is an enzyme that helps erythrocytes maintain metabolic processes despite injurious conditions
Certain drugs, foods, infection fever, acidosis, hypoxaemia act as stressors
Asymptomatic unless stressors are present

57
Q

Hereditary spherocytosis

A

Autosomal dominant trait
Abnormality of proteins or spectrins of the erythrocyte membrane leading to an increased concentration of intracellular sodium
Causes splenomegaly and microcytic spherocytes
Haemolytic anaemias result

58
Q

Sickle cell disease in children

A

Disorders characterized by presence of an abnormal hemoglobin (Hgb S)
Deoxygenation and dehydration cause the red cells to solidify and stretch into an elongated sickle shape

59
Q

Sickle cell disease can result in:

A

Vaso-occlusive crisis (thrombotic crisis), aplastic crisis, sequestration crisis, and hyperhemolytic crisis

60
Q

Sickle cell trait

A

Child inherits Hgb S from one parent and Hgb A from another

61
Q

Thalassemias

A

Autosomal recessive disorders
Synthesis of the globin chains of the hemoglobin molecule is slowed or defective
Major—homozygous inheritance
Minor—heterozygous inheritance

62
Q

Hemophilias

A

Serious bleeding disorders

Hemophilia A (classic hemophilia-factor VIII deficiency)
Hemophilia B (Christmas disease-factor IX deficiency)
Hemophilia C (factor XI deficiency)
von Willebrand disease (factor VIII deficiency)
63
Q

Congenital hypercoagulability and thrombosis

A 
Thrombophilia (increase of platelets)
Protein C deficiency
Neonatal purpurea fulminans (usually fatal syndrome)
Protein S deficiency
Antithrombin III (AT III) deficiency
64
Q

ANTIBODY-MEDIATED HAEMORRHAGIC DISEASE

A

Idiopathic thrombocytopenic purpurea
Autoimmune or primary thrombocytopenic purpurea
Autoimmune neonatal thrombocytopenia
Infants delivered to mothers with ITP
Autoimmune neonatal thrombocytopenia purpurea
Autoimmune vascular purpurea

65
Q

LEUKAEMIA in children

A

Most common malignancy of childhood

80% to 85% are acute lymphoblastic leukemias

66
Q

LYMPHOMA in children

A 
Non-Hodgkin lymphoma
Nodular and diffuse
Hodgkin lymphoma
Rare in childhood
Infectious mode of transmission
Many children with Hodgkin lymphoma demonstrate a high antibody titer to Epstein-Barr virus (EBV)

Clinical Therapeutics 2 (9 decks)

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