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the hematopoietic and lymphoid systems

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leukaemia = neoplastic cells in peripheral blood. lymphoma = tumour of the ... hereditary disorders (sy Bernard-Soulier, grey-plateles sy, m. Wiskott-Aldrich) ... – PowerPoint PPT presentation

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Title: the hematopoietic and lymphoid systems


1
the hematopoietic and lymphoid systems
2
hematopathology
  • blood
  • lymphoid organs
  • central
  • bone marrow
  • thymus
  • peripheral
  • lymph nodes
  • MALT (Waldeyers ring, intestine...)
  • splenic white pulp

3
hematopathology
  • leukaemia neoplastic cells in peripheral blood
  • lymphoma tumour of the lymph node
  • hemoblastosis
  • primary bone marrow
  • leukaemia tumoriform
  • lymphomas
  • primary lymph nodes
  • lymphoma leukemic phase

4
bone marrow
5
bone marrow
  • weight cca 1,5kg
  • red (hematopoietic) x yellow (adipose)
  • structure
  • hematopoietic cells granulopoiesis
    peritrabecular, erytropoiesis a megakaryocytes
    intertrabecular and perisinusoidal
  • corroborative elements makrophages, fibroblasts,
    mastocytes, plazmocytes, lymfocytes
  • blood sinuses
  • bone trabeculas

6
diminished hematopoiesis
  • A) total diminution
  • aplastic anemie (panmyelophtisis)
  • hereditary
  • Fanconi anemia
  • AR
  • death because of infectious and bleeding
    complications
  • /- turn into AML
  • acquired
  • infectious, irradiation, use of some drugs

7
diminished hematopoiesis
  • B) selective
  • one or more of hematopoietic lineages critical is
    peripheral blood marrow could be hypercelular
    ineffective hematopoiesis

8
diminished hematopoiesis...anemia
  • 1) anemia
  • ? total circulating RBC volume, /- ?Hb and ?O2
  • hypoxia of tissues clinical symptoms

9
anemia...loss of RBC
  • a) hemorrhage blood loss anemia
  • hypovolemia ? normocytic normochromic anemia ? ?
    erytropoiesis (bone marrow) ? reticulocytosis,
    hypochromic anemia

10
anemia...hemolytic
  • b) increased rate of RBC destruction the
    hemolytic anemias
  • anemia reactive hyperplastic erytropoiesis
  • bm ?erytropoiesis/myelopoiesis, gaucheroid cells
  • /- extramedullary hematopoiesis
  • Hb -emia, -uria

11
anemias..hemolytic..intrinsic
  • I) intrinsic (intracorpuscular) abnormalities of
    RBC
  • hereditary
  • 1) disorders of RBC membrane cytoskeleton
  • spherocytosis
  • erythrocytes spheroidal, less deformable and
    vulnerable to splenic sequestration and
    destruction
  • AD
  • anemia, splenomegalia a hemolytic icterus

12
anemias..hemolytic..intrinsic
  • 2) RBC enzyme deficiencies
  • 3) disorders of Hb synthesis hemglobin
  • deficient globin synthesis thalassemia syndromes
  • lack of or decreased synthesis of globin
    chains a chains a thalassemia
    ß chains ß
    thalassemia
  • ? synthesis of Hb ? anemia (microcytic
    hypochromic) excess of a chains in ß
    thalassemia ? insoluble aggregats ? damage RBC
    membrane ? reduction of plasticity ?
    phagocytosis, inefective erytropoiesis
  • heterozygous thalassemia minor
    homozygous thalassemia major

13
anemias..hemolytic..intrinsic
  • structurally abnormal globin synthesis
    (hemoglobinopathies) sickle cell anemia
  • atructurally abnormal Hb S on deoxygenation
    polymerization gelation or crystallization ?
    microvascular obstruction ? ischemic tissue
    damage ? removing in the spleen
    autosplenectomy

14
anemias..hemolytic..intrinsic
  • acquired
  • membrane defect paroxysmal nocturnal
    hemoglobinuria)
  • ?resistance against C3
  • granulocytes and plateles affected too ?
    hemolysis, /- trombotic complications and ?
    susceptibility to infections

15
anemias..hemolytic..extrinsic
  • II) extrinsic (extracorpuscular) abnormalities
  • 1) antibody mediated
  • isohemagglutinins
  • erythroblastosis fetalis
  • Rh (mother Rh-, father and child Rh)
  • antibodies against fetal RBC
  • hydrops fetus universalis, mental retardation,
    ?extramedulary hematopoiesis

16
anemias..hemolytic..extrinsic
  • autoantibodies
  • idiopathic (primary), drug associated, SLE
  • Coombs tests

17
anemias..hemolytic..extrinsic
  • 2) mechanical trauma to RBCs
  • mikroangiopathic hemolytic anemias
  • DIC, TTP
  • mechanic traumatization of erythrocytes
  • dialysis, valves prosthesis
  • 3) infections (malaria)

18
anemia...impaired RBC production
  • c) diminished erythropoiesis
  • 1) combination with the others in aplastic
    anemia
  • 2) pure erytroblastophtisis
  • Blackfan-Diamond syndrom
  • children
  • thymomas and T-CLL
  • 3) myelophtisic anemia
  • extensive replacement of the marrow by tumours or
    other lesions ? extramedulary hematopoiesis,
    leukoerythroblastosis

19
anemia...impaired RBC production
  • 4) iron deficiency anemia
  • most common
  • mikrocytar hypochromic
  • ?low intake (diets, malabsorptions) x ? demands
    (pregnancy, infancy, chronic blood loss)
  • gross hypoxic myocardial steatosis
  • marrow normal or hyperplastic erythropoiesis,
    decline in serrum ferritin and depletion of
    stainable iron in the bone marrow

20
anemia...impaired RBC production
  • 5) megaloblastic anemia
  • disturbance of proliferation and differentiation
    of erythroblasts ? megaloblasts, megakaryocytes
  • nuclear-cytoplasmic asynchrony
  • giant metamyelocytes ? hypersegmented neutrophils
  • ineffektive erythropoiesis
  • folate (folic acid) deficiency anemia
  • tetrahydrofolate
  • neurologic abnormalities do not occur

21
anemia...impaired RBC production
  • pernicious anemia
  • vitamin B12 (cobalamin) deficiency
  • diet, ?intrinsic faktor (parietal gastric cells),
    terminal ileum
  • gross atrophic glossitis, gastritis,
    demyelinization

22
anemia...impaired RBC production
  • 6) lack of erythropoietin
  • kidney failure, parvovirosis (B19)

23
diminished hematopoiesis... leukopenia
  • 2) leukopenia
  • a) lymfopenia
  • hereditary immunity disorders, infections(viral),
    chronical diseases, steroid therapy

24
leukopénie
  • b) neutropenia (granulocytopenia)
  • increased susceptibility to infections
  • marrow failure (aplastic anemia) ?
    agranulocytosis
  • inadequate or ineffective granulopoiesis certain
    drugs benzen, purin and pyrimidin analogs,
    anthracyklin x idiosyncrastic reaction
    (chloramfenikol, chlorpromazin, fenylbutazon)
  • accelerated removal or destruction of
    neutrophils hypersplenism, certain drugs
  • bm depend on the underlying basis ? or ?
    granulopoiesis and /- reaction to infection

25
increased hematopoiesis
  • transitory increasing of hematopoiesis
  • 1) ?erythropoiesis polycythemia
  • increased erythropoietin levels
  • appropriate lung disease, high-altitude living,
    cyanotic heart disease
  • inappropriate erythropoietin-secreting tumours,
    doping
  • bm hypercellular, inappropriate increasing of
    erythropoiesis
  • no extramedullary hematopoiesis!

26
increased hematopoiesis
  • 2) leukocytosis
  • a) lymfocytosis chronical infections (IM)
  • b) granulocytosis acute bacterial infections
    (pyogenic organisms), sterile inflammation
    (tissue necrosis, burns) ? leukemoid reaction
    (like in CML)
  • c) eosinophilia allrgic disorders, parasitic
    infestation, drug reaction, certain mlg
  • 3) thrombocytosis infections, chronical
    bleeding, tumours, iron deficiency

27
myelodysplastic syndromes
  • heterogeneous group of disorders
  • some evidence of bone marrow failure and
    dysplasia in one or more myeloid cell lineages
  • may evolve to AML
  • chromosomal aberrations
  • primary x secundary (radiotherapy, alkylating
    agent therapy)
  • bm hypercellular, ? erythropoiesis, morphological
    changes, /- fibrosis

28
myelodysplastic syndromes...histological
classification
  • refractory anemia (RA)
  • refractory anemia with ring sideroblasts (RARS)
  • refractory cytopenia with multilineage dysplasia
  • refractory anemia with excess blasts (RAEB)
  • MDS, unclassifiable

29
chronic myeloproliferative diseases
  • CMPDs clonal haematopoietic stem cell disorders
    characterised by proliferation in the bone marrow
    of one or more of the myeloid (i.e. granulocytic,
    erythroid and megakaryocytic) lineages

30
CMPD
  • A) chronic myelogenous leukaemia
  • most common
  • adults, 30-60eyars
  • neutrophilic leukocytosis in peripheral blood
  • Ph t(922) Philadelphia chr.
  • bm hypercellular (?granulopoiesis,
    ?megakaryocytes), /- fibrosis
  • extramedullary leukaemic infiltration spleen,
    liver
  • ? accelerated phase ? blast phase

31
CMPD
  • B) polycythaemia vera (polycythaemia rubra vera,
    m. Vaquez-Osler)
  • ? erythropoiesis
  • hypertension, thrombosis, haemorrhage
  • bm
  • initial phase hypercellular, with increased
    erythropoiesis extramedullar infiltration ?
    hepatosplenomegaly
  • /- blast phase or spent phase

32
CMPD
  • C) essential thrombocythaemia
  • proliferation primarly magakaryocytic lineage
  • sustained thrombopcytosis in the blood
  • bm large, mature megakaryocytes
  • D) chronic idiopathic myelofibrosis
  • proliferation of mainly megakaryocytes,
    associated with reactive deposition of bone
    marrow connective tissue and extramedullary
    hematopoiesis

33
acute leukaemias
  • causes
  • complication of certain chromosomal disease (m.
    Down, Fanconiho anémie, Klinefelteruv syndrom...)
  • radiation
  • chemicals (benzen, alkylating agents, drugs)
  • viruses (HTLV-1)
  • AML, ALL
  • symptoms combination of aplastic anemia and
    agranulocytosis
  • bm leukaemic infiltration, /- extramedullar
    infiltration (liver, spleen, kidney, CNS)
  • myelosarcoma (chloroma)

34
acute myeloid leukaemias... histological
classification
  • M0...acute myeloblastic l. minimally
    differentiated
  • M1 ...acute myeloblastic l. without maturation
  • M2...acute myeloblastic l. with maturation
  • M3...acute promyelocytic l.
  • M4...acute myelomonocytic l.
  • M5...akutní monocytic l.
  • M6...acute erythroid l.
  • M7...acute megakaryoblastic l.

35
acute lymphoblastic leukaemias... histological
classification
  • precursor B- and T- cell lymphoblastic
    leukaemia/lymphoblastic lymphoma

36
proliferation of macrophages, histiocytosis
  • A) reactive proliferation of macrophages
  • bone marrow, many causes (hemosiderosis, aiha,
    viral infections)
  • lysosomal storage diseases (m. Gaucher,
    Niemann-Pick...)

37
proliferation of macrophages, histiocytosis
  • B) hemofagocytic syndroma
  • ? proliferation of macrophages or histiocytic
    precursores ? haemofagocytosis ? cytopenia
  • hepatosplenomegaly, fever
  • proliferating macrophages clonal (mlg
    histiocytosis) x reaction (infection, Kawasaki, T
    lymphomas)
  • fatal haemofagocytosis

38
proliferation of macrophages, histiocytosis
  • C) histiocytosis X (Langerhans cells
    histiocytosis)
  • 1) solitary eosinophilic granuloma
  • bng
  • bones (unifocal lytic lesion), skin, lymph nodes,
    lungs
  • Langerhans cells (birbeck granules)
    eosinophils, /- plasma cells and lymphocytes
  • 2) m. Hand-Schüler-Christian
  • trias multifocal lytic lesions of bone
    exophtalamus diabetes insipidus

39
proliferation of macrophages, histiocytosis
  • 3) m. Abt-Letterer-Siwe
  • mlg
  • children before 2 years of age
  • cutaneous lesions resembling seborrheic skin
    eruptions hepatosplenomegaly, lymphadenopathy,
    pulmonary lesions, osteolytic bone lesions ?
    anemia and thrombocytopenia, reccurent infections

40
metastasis
  • osteolytic x osteoplastic
  • prostate, breast, stomach, lung cancer

41
bone marrow necrosis
  • ischemia
  • vascular collaps in hypercellular marrow
  • metastatic obstruction
  • sickle cell disease, DIC...
  • symptoms pain, fever, hematopoietic precursors
    in peripheral blood

42
transplantation
  • transplantation bone marrow, peripheral stem
    cells
  • autologous x allogenneous (relatives,
    non-relatives)
  • indications
  • hematological tumours, immunodeficiency,
    anemias, b.m. aplasia
  • non-hematological tumour metastasis

43
transplantation
  • bone marrow suppression ? graft
  • hypocellularity ? proliferation
  • immunosuppression!
  • GvHD acute x chronic
  • skin, intestine, liver

44
bleeding disorders
  • cause
  • defect in the vessel wall
  • platelet deficiency or dysfunction
  • derangement of coagulation factors

45
bleeding disorders...vascular
  • A) defects in the vessel wall
  • 1) hereditary
  • a) m. Osler-Rendu-Weber (hereditary hemorhagic
    teleangiektasias)
  • capillary aneurysms in the skin and mucous
    membranes
  • b) connective tissue disorders
  • m. Ehlers-Danlos
  • Marfans syndrome

46
bleeding disorders...vascular
  • 2) acquired
  • a) avitaminosis C, ? kortikosteroids
  • cutaneous, intramuscular, mucosal bleeding
  • b) purpura Henoch-Schönlein
  • circulating IC ? skin, kidney

47
bleeding disorders...plateles
  • B) plateles deficiency or dysfunction
  • 1) thrombocytopenia
  • a) decresed production
  • aplastic anemia
  • hereditary disorders (sy Bernard-Soulier,
    grey-plateles sy, m. Wiskott-Aldrich)

48
bleeding disorders...plateles
  • b) increased destruction
  • splenomegaly, arteficial valves,...
  • DIC (disseminated intravascular coagulation)
  • activation of the coagulation sequence, leading
    to formation of thrombi throughout the
    microcirculation ? consumption of plateles and
    coagulation factors and secondarily activation of
    fibrinolysis

49
bleeding disorders...plateles
  • thrombotic thrombocytopenic purpura (TTP)
  • thrombocytopenia, fever, microvessel obstruction
    symptoms
  • ? microangiopathic hemolytic anemia
  • hyaline thrombi in the microcirculation
  • hemolytic-uremic syndrome (HUS)
  • E.coli
  • kidney cortex necrosis, intestinal bleeding

50
bleeding disorders...plateles
  • idiopathic thrombocytopenic purpura (ITP)
  • autoimmune origin
  • destruction in the spleen ? splenectomy
  • bm /- increased megakaryopoiesis

51
bleeding disorders...plateles
  • 2) platelet dysfunction
  • adhesion disorder (Bernard-Soulier, m. von
    Willebrand)
  • aggregation disorder (thrombasthenia Glanzmann)
  • secretion disorder tromboxan A2 inhibition
    (aspirin)

52
bleeding disorders...coagulation factors
  • C) coagulation disorders
  • 1) hereditary deficiencies
  • a) hemophilia A (classic hemophilia)
  • f VIII (severe activity lt 1!)
  • X chromosoma (new mutation x familiar)
  • easy bruising and massive hemorrhage after trauma
    or operative procedures, spontaneous
    hemorrhages joints (hemarthroses) ? progressive
    deformities
  • b) hemophilia B (Christmas disease)
  • f IX

53
bleeding disorders...coagulation factors
  • 2) acquired
  • a) DIC
  • b) liver diseases
  • synthesis of coagulation factors (fibrinogen,
    prothrombin, fV, VII, IX-XI) anticoagulation
    and fibrinolytic factors
  • c) vitamin K
  • food, synthesis in the large intestine
    (bacterias)
  • d) anticoagulation therapy

54
lymph vessels and nodes
55
lymphatic vessels
  • A) lymphoedema
  • lymph is protein-rich ? lymphostasis leads to
    fibroproduction, /- infectious and ulcerative
    complications
  • 1) hereditary Milroys disease
  • valvular disorder
  • 2) acquired lymphoedema
  • lymphoedema praecox
  • secondary lymphoedema obstruction and
    lymphostasis (mlg, inflammatory changes)

56
lymphatic vessels
  • B) lymphangiectasia
  • focal extension of lymphatic vessels
  • skin, small intestine (chylangiectasia)
  • ? lymforhea (chylothorax...)

57
lymphatic vessels
  • C) lymphangiitis
  • lymph vessels draining the primary (infectious)
    focus
  • ß hemolytic streptococci
  • regional lymphadenitis
  • clinical red subcutaneous line
  • histology
  • lymphangiitis simplex
  • lymphangiitis purulenta pus fibrin ? spreading
    ? abscesses, trombophlebitis

58
lymfatic nodes...structure
  • cells lymphocytes, dendritic cells (FDRC, IDRC),
    macrophages with apoptotic bodies, NK cells
  • follicles B zone
  • lymphocytes from the bm ? primary follicle ?
    immunity stimulation ? germinal centres
    secondary follicle, immunity answer ?
    polarization of germinal centres
  • germinal centres B cells augmentation, selection
    Ag high affinity clones ? plasma cells
    differentiation ? migration into medulla, waiting
    to secondary immunity answer

59
lymph nodes...structure
  • medulla
  • lymphatic tissue between medullar sinuses
  • small lymphocytes, plasma cells
  • paracortex T zone
  • mainly CD4 T cells, small venules
  • T lymphocytes 70 of lymphocytes in lymph node
    and 80-90 in blood
  • sinuses
  • incoming lymph vessels ? subcapsular (marginal)
    sinus ? interfollicular ? medulla ? outgoing
    vessels

60
lymph nodes...regressive changes
  • A) regressive changes and circulatory disorders
  • 1) infarction
  • vasculitis
  • tumorous infiltration
  • vascular transformation of sinuses
  • 2) atrophy
  • lipomatous
  • hyalin
  • 3) pigmentation
  • 4) amyloidosis
  • 5) storage diseases

61
lymph nodes...inflammation
  • B) lymphadenitis
  • 1) acute nonspecific
  • inflammation of regional lymph node
  • clinicaly enlarged, erythematous lymph nodes
  • histology ? follicles, mitoses, sinuses filled
    with granulocytes, histiocytes
  • /- healing with fibrous scarse

62
lymph nodes...inflammation
  • 2) chronic nonspecific lymphadenitis
  • etiology
  • a) follicular hyperplasia
  • etio tonsillitis, respiratory infections, RA,
    syphilis, AIDS
  • histology ? germinal centers, fanciful shapes,
    many mitoses, blastic forms of cells could be
    misinterpreted like mlg lymphoma!

63
lymph nodes...inflammation
  • progressive transformation of germinal centres
  • connection with HD (paragranuloma)
  • m. Castleman (angiofollicular hyperplasia)
  • lolly pops follicles
  • unifocal bng x multifocal fatal

64
lymph nodes...inflammation
  • b) paracortical hyperplasia
  • etio viruses (IM, HSV), inoculation, some drugs
  • histology enlarged paracortex, with many IDRC,
    small follicles in the periphery of the lymph
    node, T imunoblasts
  • c) reactive sinusoidal histiocytosis
  • etio reactive (different Ag)
  • histology dilated sinuses filled with histiocytes

65
lymph nodes...inflammation
  • m. Rosai-Dorfman (masive sinusoidal
    histiocytoses)
  • intrasinusoidal macrophages with emperipolesis
  • d) mixed reactive hyperplasia
  • etio toxoplasmosis (epitheloid granulomas)

66
lymph nodes...inflammation
  • 3) granulomatus purulent
  • epitheloid granulomas with central necrosis with
    accumulation of neutrophils
  • cat scratch disease
  • veneric lymphogranuloma (Chl.trachomatis)
  • mesenterial lymphadenitis (Y.enterocolitica)
  • ulcus molle (H. ducreyi)

67
lymph nodes...inflammation
  • 4) granulomatous necrotic
  • tularemia (Fr. tularensis)
  • plague (Y. pestis)
  • anthrax (B. antracis)
  • 5) granulomatous
  • tuberculoid granulomas without central necrosis
  • sarcoidosis, m. Crohn...
  • 6) TBC lymphadenitis
  • miliary x caseous productive

68
lymph nodes...inflammation
  • 7) granulomatous reaction to lipid materials
  • m. Whipple
  • lipid vacuoles, around epitheloid histiocytes,
    intracytoplasmic PAS material
  • 8) granulomatous reaction to foreign bodies
  • silicic material in prosthesis

69
lymph nodes...neoplasms
  • C) neoplasms, malignant lymphomas
  • 1) m. Hodgkin (HD)
  • group of disesases
  • presence of distinctive neoplastic giant cells
    Reed-Sternberg cells, Hodgkin cells, admixed with
    a variable infiltrate of reactive, nonmalignant
    inflammatory cells
  • young people

70
lymph nodes...neoplasms...HD
  • classification
  • nodular lymphocytic predominance Hodgkin lymphoma
  • classic
  • lymphocyte rich HL (LR-CHL)
  • nodular sclerosis (NS-CHL)
  • mixed cellularity (MC-CHL)
  • lymfocyte depleted (LD-CHL)

71
lymp nodes...neoplasms...NHL
  • 2) non Hodgkin lymphomas
  • predominance of neoplastic cells
  • elder patients
  • B and T cells

72
lymph nodes...neoplasms...B-NHL
  • a) B-NHL
  • chronic lymphocytic leukaemia/small lymphocytic
    lymphoma (CLL/SLL)
  • follicular lymphoma (FCL)
  • mantle cell lymphoma (MCL)
  • marginal zone lymphoma (MZL) SMZL, ENMZLMALT,
    NMZL
  • hairy cell leukaemia (HCL)
  • diffuse large B-cell lymphoma (DLBCL)
  • Burkitt lymphoma

73
lymph nodes...neoplasms...B-NHL
  • plasma cell neoplasms
  • monoclonal gammopathy of undetermined
    significance (MGUS)
  • lymphoplasmacytic lymphoma (LPL), m. Waldenström
  • plasmacytoma
  • multiple myeloma

74
lymph nodes...neoplasms...T-NHL
  • b) T-NHL
  • peripheral T cell lymphoma (PTL)
  • anaplastic large T cell lymphoma (ALCL)
  • angioimmunoblastic T cell lymphoma (AILT)
  • adult T cell leukaemia
  • mycosis fungoides/Sezary syndrom

75
spleen
76
spleen
  • structure
  • white pulp lymphoid tissue
  • red pulp venous sinuses ? hilus
  • splenomegaly venosthasis, inflammation,
    neoplasms
  • hypersplenism increased function ? cytopenia
  • hyposplenism ? susceptibility to certain
    bacterial infections

77
spleen...regressive changes, circulatory disorders
  • A) inborn anomalies
  • accessory spleens spleniculi
  • B) regressive changes and circulatory disorders
  • 1) amyloidosis
  • secondary (AA)
  • /- hyposplenism

78
spleen...regressive changes, circulatory disorders
  • 2) storage diseases
  • 3) hemolytic anemias
  • ? splenic function ? splenomegaly
  • hereditary spherocytosis
  • 4) chronic perisplenitis
  • 5) infarkt sleziny
  • white (embolization, vasculitis)
  • red (thrombosis of lienal vein)

79
spleen...regressive changes, circulatory disorders
  • 6) chronic venosthasis
  • 7) splenic rupture, bleeding
  • traumatic

80
spleen...inflammation
  • A) inflammation
  • 1) acute septic tumour
  • reaction to general infection x tumour lysis
  • clinicaly tense capsula, soft tissue
  • histology red pulp cellular, small abscesses
    (central pyemia)

81
spleen...inflammation
  • 2) chronic inflammatory tumour
  • chronic infections (IE)
  • histology red pulp hyperemia, reactive
    hyperplasia of the white pulp
  • malaria
  • TBC, histoplasmosis, leishmaniosis,
    trypanosomiasis
  • AIDS

82
spleen...tumours
  • D) tumours and pseudotumours
  • 1) cystic formations
  • posttraumatic pseudocysts
  • parasitary cysts
  • /- dermoid cyst
  • 2) hamartoma (splenoma)
  • nodule
  • histology chaotic sinuses a fibrus tissue
    incorrect arrangement of the red pulp
  • /- hypersplenism

83
spleen...tumours
  • 3) hemangioma
  • histology cavernous blood spaces, thrombosis
  • 4) littoral cell angioma
  • phagocytosis ? pancytopenia
  • 5) inflammatory pseudotumour
  • histology inflammatory cells fibroproduction

84
spleen...tumours
  • 6) malignant lymphomas
  • primary SMZL
  • secondary
  • more often
  • secondary infiltration by NHL, HD, CML, HCL
  • 7) epithelial metastasis
  • microscopically

85
thymus
86
thymus...structure
  • structure
  • lobulus
  • cortex and medulla mixture of T lymphocytes and a
    epithelial cells lymphoepithelial organ
  • cortex mainly T lymphocytes
  • lymphatic follicles without germinal centres
  • medulla thymocytes, Hassal bodies

87
thymus...function
  • function
  • production of small lymphocytes with cellular
    immunity
  • TdT a CD1 ? maturation ? CD4 a CD8 ? postthymic
    lymfocytes in medulla CD4 (helpers/inducers),
    CD8 (suppressor/cytotoxic), loss of TdT a CD1 ?
    blood, peripheral lymphatic organs
  • main role intrauterine and in childhood

88
thymus...dysgenesis
  • A) thymic dysgenesis
  • primary immunodeficiency syndromes (diGeorge,
    Nezelof...)

89
thymus...regressive changes
  • B) regressive changes
  • 1) lipomatous atrophy (involution)
  • puberty involution with increase of adipous
    tissue ? thymocytes, calcification of Hassal
    bodies...
  • 2) acute (accidental) involution
  • etio corticosteroids stress
  • histology fragmentation of cortical thymocytes,
    cystic transformation of Hassal. bodies,
    lymfocytes disappeared, in cortex only spindle
    epithelial cells

90
thymus...hyperplasia
  • C) thymic hyperplasia
  • primary hyperplasia
  • myasthenia gravis
  • histology lymphoid hyperplasia, lymphatic
    follicles with germinal centres
  • Ab anti acetylcholin-receptors

91
thymus...neoplasms
  • D) neoplasms
  • 1) thymomas
  • epithelial thymic cells lymphocytes
  • local manifestation
  • association with myasthenia gravis
  • 2) neuroendocrinne tumors
  • 3) germinal cells tumours (teratoma, seminoma)
  • bng, cystic
  • 4) malignant lymphoma
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