Hematolymphoid 2 Flashcards

1
Q

How is iron absorbed, transported, stored

A
  • Absorbed in small intestine → bound to transferrin and transported → incorporated into hemoglobin
  • Stored in ferritin (aggregates as hemosiderin)
  • Aging RBCs destroyed in the spleen and iron reutilized
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2
Q

Causes of iron deficiency anemia

A
  • Decreased intake
  • Decreased absorption
  • Increased loss
  • Increased requirements
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3
Q

Iron deficiency anemia lab results

A
  • Blood smear: hypochromic microcytic anemia
  • Hemoglobin and hematocrit low
  • Serum iron and ferritin low
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4
Q

Aplastic anemia

A

Characterized by loss of multipotent stem cell resulting in pancytopenia
- Decreased RBC = anemia
- Decreased WBC = infection prone
- Decreased platelets = bleeding

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5
Q

Aplastic anemia causes

A

Primary: Idiopathic

Secondary (related to BM suppression):
- Chemical agents (cytotoxic drugs)
- Radiation
- Viral infection
- Inherited

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6
Q

Megaloblastic anemia

A

Caused by deficiency of vitamin B12 or folic acid (essential for DNA synthesis and blood cell production)

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7
Q

Megaloblastic anemia pathogenesis

A
  • Deficiencies delay normal RBC maturation
  • RBC precursors do not mature and are transformed into megaloblasts
  • Megaloblasts are destroyed in BM or spleen
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8
Q

Megaloblastic anemia diagnosis

A

Diagnosis
- Peripheral blood smear: oval macrocytes, large segmented neutrophils
- BM biopsy: hypercellular with megaloblasts

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9
Q

Causes of Vitamin B12 deficiency

A
  • Decreased intake (uncommon)
  • Impaired absorption/increased loss (Pernicious anemia)
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10
Q

Pernicious anemia pathogenesis

A

Atrophic gastritis → results in decrease in gastric parietal cells → insufficient IF → B12 deficiency → megaloblastic anemia

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11
Q

Results of folate deficiency

A

Results in megaloblastic anemia (similar characteristics as B12 deficiency)

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12
Q

Causes of folate deficiency

A
  • Impaired absorption/increased loss (Disease of duodenum)
  • Decreased intake
  • Increased requirement
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13
Q

Thalassemias

A

Genetic defect in synthesis of normal Hb (no abnormal Hb is produced, defect is quantitiative)

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14
Q

Thalassemia classifications

A

Classified by type of chain affected
- a thalassemias → defecetive a chain synthesis
- b thalassemias → defecetive b chain synthesis

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15
Q

a thalassemia

What types?

A
  • Single gene deletion → silent carrier (asymptomatic)
  • 2 gene deletion → a thalassemia trait (mild anemia)
  • 3 gene deletion → Hemoglobin H disease (moderate to severe anemia; HbH tetramer of B globulins forms → poor O2 delivery → HbH sequestering/destruction in spleen)
  • 4 gene deletion → Hydrops fetalis (most severe; excess y chains form tetramers → poor O2 delivery → fetus death)
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16
Q

B thalassemia

What types?

A
  • Thalassemia minor (heterozygote) → mild, nonspecific symptoms
  • Thalassemia major (hommozygote) → severe, decreased RBC production and unpaired a chains prone to hemolysis/precipitation
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17
Q

Sickle cell disease

A

Group of inherited disorders of Hb due to defect in B globin gene
- In low O2 states, abnormal Hb polymerizes making RBCs sickle
- Results in chornic hemolytic anemia and small vessel occlusion

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18
Q

Cause of sickle cell disease

A
  • Point mutation in B globin gene → aa substitution → abnormal B chain
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19
Q

Sickle cell anemia

A

Homozygous for defective gene
- HbS < 40% → asymptomatic
- HbS 40-80% → mild to moderate
- HbS 80% → severe

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20
Q

Sickle cell trait

A

Heterozygous for defective gene
- <40% Hb is Hbs, thus cells do not sickle and patients are asymptomatic

21
Q

Hereditary spherocytosis

A

Group of genetic defect characterized by defect of structural proteins in RBC (most are autosomal dominant)
- Defective RBCs unable to maintain biconcave shape (spheres) and are removed by the spleen

22
Q

Clinical findings of hereditary spherocytosis

A

Anemia, splenomegaly, jaundice

23
Q

Polycythemia

A

(Erythrocytosis) → An increase in the number of RBCs

24
Q

Relative polycythemia causes

A

Hemoconcentration (dehydration)

25
Absolute polycythemia causes
Primary (low erythropoietin): - Polycythemia rubra vera (uncontrolled production of RBCs due to myleoproliferative disorder) Secondary (high erythropoietin): - Living at high altitude; chronic lung disease
26
Polycythemia symptoms
Increased viscosity (sluggish blood flow and tendency to clot)
27
Leukocytosis
Increased number of WBCs - Typically benign - Often associated with splenomegaly or lymphadenopathy
28
Types of leukocytosis and effects
Neutrophilic leukocytosis (increased neutrophils) → acute bacterial infections Eosinophilic leukocytosis (increased eosinophils) → parasites, allergies, drug reaction Lymphocytosis (increased lymphocytes) → viral infections, chronic infections (e.x. TB)
29
Leukopenia
Reduction in WBC count
30
Types of leukopenia
Neutropenia and lymphopenia
31
Neutropenia (Leukopenia)
Decreased number of neutrophils → susceptible to bacterial infection
32
Neutropenia (Leukopenia) Causes
- Ineffective granulopoiesis (e.x. aplastic anemia) - Accelerated removal or destruction of neutrophils (e.x. Ab destruction by SLE)
33
Lymphopenia (Leukopenia)
Decreased number of lymphocytes (selective lymphopenia = decreased subset of lymphocytes)
34
Lymphopenia (Leukopenia) causes
- Ineffective hematopoeisis - Accelerated removal or destruction of lymphocytes
35
Acute Lymphoblastic Leukemia
- Prevelance: 20% (most common type in children) - BM infiltrated with immature lymphoid cells spill over into blood - Lethal without chemotherapy
36
Acute Myelogenous Leukemia
- Prevelance: 40% (most common type in adults) - BM infiltrated by immature myeloid cells spill over into blood - Classified by WBC linneages affected - Lethal without treatment (chemotherapy, radiotherapy, BM transplant)
37
Chronic Myelogenous Leukemia
- Prevelance: 15% (incidence increases with age) - BM infiltrated by myeloid cells - Characterized by Philadelphia chromosome - Slow insidious onset, asymptomatic - 3 phases: chronic phase → accelerated phase → blast crisis
38
Chronic Lymphocytic Leukemia
- Prevalence: 25% (Elderly, slow progression) - BM infiltrated by lymphoid cells, spills into blood - Involves LNs/spleen → small lymphocutic lymphoma - CLL cells grow slowly
39
Hodgkin Lymphoma
- Biomodal age distribution (25; 55) - 5 types - Staging is key for prognosis - Chemotherapy effective
40
Hodgkin lymphoma types
Classical HL (90%) (presence of Reed Sternberg cell → malignant B cell): - Nodular slcerosis HL (70%) - Mixed cellularity HL (20%) - Lymphocyte-rich HL (5%) - Lymphocyte-depleted HL (5%) Nodular lymphocyte predominant of HL (10%)
41
Multiple myeloma age group
Middle age (>45)
42
Multiple myeloma
Malignant disease of plasma cells - Plasmacytoma = single lesion (can be of bone or extramedullary (soft tissue))
43
Multiple Myeloma pathogenesis
Malignant transformation of single plasma cell → clonal expansion → monoclonal expansion of plasma cells → secrete monoclonal Ig's
44
Multiple myeloma clinical features
CRAB: - C = Calcium (Hypercalcemia → calcium released from destroyed bones) - R = Renal failure (Ig's excreted damage renal tubes; hypercalcemia damages kidneys) - A = Anemia (replacement of normal BM by tumor) - B = Bone lesions (lytic bone lesions caused by plasma cells)
45
Bleeding Disorders
Vascular disorders or Platelet disorders
46
Vascular disorders (Bleeding disorders)
- Common cause: mechanical trauma - Vessel wall fragility (old age, connective tissue disorder) - Immune damage (vasculitis)
47
Types of platelet disorders (Bleeding disorders)
Qualitative or quantitative
48
Quantitative platelet disorder (bleeding disorders)
- Decreased production (aplastic anemia) - Increased destruction (autimmune disorders) - Increased utilization (disseminated intravascular coagulation)
49
Qualitative platelet disorder (bleeding disorders)
Acquired: NSAIDs and aspirin Congenital: - Defect platelet adhesion (Benard-Souilver syndrome) - Defective platelet aggregation (Glanzmann thrombasthenia)