thymic and splenic disorders - Zaloga Flashcards

1
Q

thymus

A
  • lymphepithelial organ
  • cell mediated immunity
  • cortex –> developing T cells, Macs
  • medulla –> immunocompetent T cells, hassals, Macs, DCs
  • hassals corpuscles –> form whirl bodies; desmosomes for thymus-blood barrier**
  • myoid cells (in medulla) move lymphocytes through maturation –> associated with myasthenia gravis
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2
Q

myasthenia gravis (graves disease)

A

-autoantibodies against ACh receptor

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3
Q

DiGeorge syndrome

A
  • thymic developmental disorder
  • thymic hypoplasia –> defects in cell mediated immunity**
  • 22q11 deletion syndrome
  • ventricular septal defects
  • cleft palate
  • hypoparathyroidism –> low Ca2+ (tetany) and high phosphate
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4
Q

thymic hyperplasia

A
  1. true hyperplasia
    - increase size, weight
    - increase lymphocyte #
    - normal cortex, medulla
  2. thymic follicular hyperplasia
    - expansion of B cells in follicles (usually only small # of B cells)
    - present with myasthenia gravis
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5
Q

thymomas

A
  • tumor of thymic epithelial cells –> impinge on mediastinal structures
  • thymocytes are CD4+ and CD8+ T cells
  • cortical thymomas with autoimmune disease
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6
Q

types of thymomas

A
  1. noninvasive: usually medullary type, sparse thymocyte infiltrates
  2. mixed: 50% of thymomas, benign appearing medulla, polygonal cortical type, more likely invasive**, denser thymocyte infiltrates
  3. invasive: 25% of thymomas, benign appearing cortex, most invasive and aggressive
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7
Q

thymic carcinoma

A
  • 5% of thymomas
  • show dysplasia and invasiveness
  • sometimes metastasize to lung
  • most squamous cell carcinoma
  • some contain EBV genomes
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8
Q

spleen

A
  • mononuclear phagocyte system
  • usually 10-11 cm
  • site for immune response to blood-borne antigens and filter
  • red pulp –> blood
  • white pulp –> lymphocytes
  • T cells in PALS, B cells in germinal centers
  • sinusoids (filter and antigen detector) carry blood through splenic cords (cords of billroth) –> APCs and Macs phagocytize blood**
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9
Q

spleen functions

A
  1. phagocytose senescent RBCs (trapped in cords)
  2. antibody production: DCs present antigen to T cells –> make plasma cells
  3. extra medullary hematopoiesis (in anemia or marrow dysfunction)
  4. sequestration of blood cells if enlarged –> cytopenias
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10
Q

splenomegaly

A
  • responds to systemic inflammation by increasing activity

- hypersplenism syndrome –> cytopenias

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11
Q

splenic insufficiency/asplenia

A
  • caused splenectomy or autoinfarction
  • increased risk of sepsis and encapsulated organisms***
  • sickle cell patients need vaccination**
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12
Q

nonspecific acute splenitis

A
  • due to blood-borne infection

- infiltration of neutrophils etc. into germinal centers causing inflammation –> necrosis or infarction of white pulp

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13
Q

congestive splenomegaly

A
  • Caused by chronic venous outflow obstruction with portal or splenic vein hypertension
  • sequestering of cells (sinusoids filled with blood cells)

-obstruction causes: liver cirrhosis (most common), portal vein thrombosis, systemic venous congestion in right side of heart

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14
Q

splenic rupture

A
  • cause: trauma
  • spontaneous ruptures due to infectious mono, typhoid fever, malaria, lymphoid neoplasm
  • emergency due to internal bleeding –> splenectomy
  • avoid contact sports with mono**
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15
Q

splenic infarcts

A
  • lesions due to occlusion of splenic artery and its branches
  • usually due to noninfectious emboli from the heart** (common in enlarged spleens)
  • forms depression, more pale (if older) or hemorrhagic (if recent)
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16
Q

spleen disorders

A
  1. neoplastic involvement of myeloid and lymphoid tumors most common**
    - lymphangiomas and hemangiomas usually
17
Q

spleen disorders - congenital anomalies

A
  1. asplenia (rare) –> associated with situs inversus and cardiac malformation
  2. hypoplasia (common) –> due to incomplete splenic development or splenic infarct
  3. accessory spleens (35%) –> benign and asymptomatic, same structure and function as normal spleen
    - seen in hereditary spherocytosis and ITP
    - splenectomy may not improve symptoms if accessory spleens present