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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
Lymphocyte
↑ acute viral infection; ↓ drug destruction, viral destruction
26
INFECTIOUS MONONUCLEOSIS
Acute, self-limiting infection of B lymphocytes transmitted by saliva through personal contact * Commonly caused by the Epstein-Barr virus (EBV)—85%
27
Symptoms of infectious mononucleosis
Symptoms: fever, sore throat, swollen cervical lymph nodes, increased lymphocyte count, and atypical (activated) lymphocytes
28
Diagnostic Test for infectious mononucleosis
Monospot qualitative test for heterophilic antibodies
29
Leukemia
Malignant disorder of the blood and blood-forming organs | Excessive accumulation of leukemic cells
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Acute Leukemia
Presence of undifferentiated or immature cells, usually blast cells
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Chronic Leukaemia
Predominant cell is mature but does not function normally
32
4 classifications for leukaemia
Lymphocytic (lymph gland) Myeloid (bone marrow) Acute (immature cells) Chronic (mature, dysfunctional cells)
33
Signs and symptoms of leukaemias
``` 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
MYELOMA
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
Multiple myeloma causes increased osteoclastic bone destruction. Clinical manifestations are:
Cortical and medullary bone loss Skeletal pain Recurring infections due to loss of the humoral immune response
36
LYMPHADENOPATHY
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
MALIGNANT LYMPHOMAS
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
HODGKINS LYMPHOMA
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
Non-Hodgkin lymphomas are linked to
Chromosome translocations, viral and bacterial infections, environmental agents, immunodeficiencies, and autoimmune disorders
40
Burkitt Lymphoma
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
SPLENIC FUNCTION
Largest of the secondary lymphoid organs Serves as blood reservoir Supplies lymphocytes for response to blood born microorganisms
42
Thrombocytopenia
``` 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
Causes of Thrombocytopenia
Hypersplenism, autoimmune disease, hypothermia, and viral or bacterial infections that cause disseminated intravascular coagulation (DIC)
44
Immune thrombocytopenic purpurea (ITP)
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
Clinical manifestations of Immune thrombocytopenic purpurea (ITP)
Petechiae and purpurea, progressing to major hemorrhage
46
Thrombotic thrombocytopenic purpurea (TTP)
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
Essential (primary) thrombocythemia
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
Alterations to platelet function
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
ALTERATIONS OF COAGULATION
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
DISSEMINATED INTRAVASCULAR COAGULATION (DIC)
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
Clinical signs and treatment of (DIC)
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
Iron deficiency anaemia in children
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
Acquired congenital hemolytic anemia
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
Hemolytic disease of the newborn (HDN)
``` Manifestations: Anemia Hyperbilirubinemia Icterus neonatorum (neonatal jaundice) Kernicterus (bilirubin deposition in the brain) ```
55
Anemia of infectious disease
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
Glucose-6-phosphate dehydrogenase deficiency (G6PD)
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
Hereditary spherocytosis
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
Sickle cell disease in children
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
Sickle cell disease can result in:
Vaso-occlusive crisis (thrombotic crisis), aplastic crisis, sequestration crisis, and hyperhemolytic crisis
60
Sickle cell trait
Child inherits Hgb S from one parent and Hgb A from another
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Thalassemias
Autosomal recessive disorders Synthesis of the globin chains of the hemoglobin molecule is slowed or defective Major—homozygous inheritance Minor—heterozygous inheritance
62
Hemophilias
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
Congenital hypercoagulability and thrombosis
``` Thrombophilia (increase of platelets) Protein C deficiency Neonatal purpurea fulminans (usually fatal syndrome) Protein S deficiency Antithrombin III (AT III) deficiency ```
64
ANTIBODY-MEDIATED HAEMORRHAGIC DISEASE
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
LEUKAEMIA in children
Most common malignancy of childhood | 80% to 85% are acute lymphoblastic leukemias
66
LYMPHOMA in children
``` 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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