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the hematopoietic and lymphoid systems. hematopathology. blood lymphoid organs central: bone marrow thymus peripheral: lymph nodes MALT (Waldeyer´s ring, intestine...) splenic white pulp . hematopathology. leukaemia = neoplastic cells in peripheral blood

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  • blood
  • lymphoid organs
    • central:
      • bone marrow
      • thymus
    • peripheral:
      • lymph nodes
      • MALT (Waldeyer´s ring, intestine...)
      • splenic white pulp
  • 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
bone marrow5
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
diminished hematopoiesis
diminished hematopoiesis

A) total diminution

aplastic anemia (panmyelophtisis)

  • hereditary:
    • Fanconi anemia
      • AR
      • death because of infectious and bleeding complications
      • +/- turn into AML
  • acquired:
    • infectious, irradiation, use of some drugs
diminished hematopoiesis7
diminished hematopoiesis

B) selective

  • one or more of hematopoietic lineages critical is peripheral blood – marrow could be hypercelular = „ineffective hematopoiesis“
diminished hematopoiesis anemia
diminished hematopoiesis...anemia

1) anemia

  • ↓ total circulating RBC volume, +/- ↓Hb and ↓O2
  • hypoxia of tissues = clinical symptoms
anemia loss of rbc
anemia...loss of RBC

a) hemorrhage: blood loss anemia

  • hypovolemia → normocytic normochromic anemia → ↑ erytropoiesis (bone marrow) → reticulocytosis, hypochromic anemia
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
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
anemias hemolytic intrinsic12

2) RBC enzyme deficiencies

3) disorders of Hb synthesis: hem+globin

  • deficient globin synthesis: thalassemia syndromes
    • lack of or decreased synthesis of globin chains: α chains = α thalassemia β chains = β thalassemia
    • ↓ synthesis of Hb → anemia (microcytic hypochromic) + excess of α chains in β thalassemia → insoluble aggregats → damage RBC membrane → reduction of plasticity → phagocytosis,inefective erytropoiesis
    • heterozygous = thalassemia minor homozygous = thalassemia major
anemias hemolytic intrinsic13
  • structurally abnormal globin synthesis (hemoglobinopathies): sickle cell anemia
    • structurally abnormal Hb S – on deoxygenation polymerization = gelation or crystallization → microvascular obstruction → ischemic tissue damage + ↑ removing in the spleen = „autosplenectomy“
anemias hemolytic intrinsic14
  • acquired
  • membrane defect: paroxysmal nocturnal hemoglobinuria)
    • ↓resistance against C3
    • granulocytes and plateles affected too → hemolysis, +/- trombotic complications and ↑ susceptibility to infections
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
anemias hemolytic extrinsic16
  • autoantibodies
    • idiopathic (primary), drug associated, SLE
    • Coombs tests
anemias hemolytic extrinsic17

2) mechanical trauma to RBCs

  • mikroangiopathic hemolytic anemias
    • DIC, TTP
  • mechanic traumatization of erythrocytes
    • dialysis, valves prosthesis

3) infections (malaria)

anemia impaired rbc production
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 → extramedullary hematopoiesis, leukoerythroblastosis
anemia impaired rbc production19
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
anemia impaired rbc production20
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
anemia impaired rbc production21
anemia...impaired RBC production
  • pernicious anemia
  • vitamin B12 (cobalamin) deficiency
  • diet, ↓intrinsic faktor (parietal gastric cells), terminal ileum
  • gross: atrophic glossitis, gastritis, demyelinization
anemia impaired rbc production22
anemia...impaired RBC production

6) lack of erythropoietin

  • kidney failure, parvovirosis (B19)
diminished hematopoiesis leukopenia
diminished hematopoiesis... leukopenia

2) leukopenia

a) lymfopenia

  • hereditary immunity disorders, infections(viral), chronical diseases, steroid therapy

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
increased hematopoiesis
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!
increased hematopoiesis26
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: allergic disorders, parasitic infestation, drug reaction, certain mlg

3) thrombocytosis: infections, chronical bleeding, tumours, iron deficiency

myelodysplastic syndromes
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 secondary (radiotherapy, alkylating agent therapy)
  • bm hypercellular, ↑ erythropoiesis, morphological changes, +/- fibrosis
myelodysplastic syndromes histological classification
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
chronic myeloproliferative diseases
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

A) chronic myelogenous leukaemia

  • most common
  • adults, 30-60eyars
  • neutrophilic leukocytosis in peripheral blood
  • Ph+ = t(9;22) = Philadelphia chr.
  • bm: hypercellular (↑granulopoiesis, ↑megakaryocytes), +/- fibrosis
  • extramedullary leukaemic infiltration: spleen, liver
  • → accelerated phase → blast phase

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

C) essential thrombocythaemia

  • proliferation primarly magakaryocytic lineage
  • sustained thrombocytosis 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
acute leukaemias
acute leukaemias
  • causes:
    • complication of certain chromosomal diseases (m. Down, Fanconi anemia, Klinefelter´s syndroma...)
    • 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“)
acute myeloid leukaemias histological classification
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...acute monocytic l.
  • M6...acute erythroid l.
  • M7...acute megakaryoblastic l.
acute lymphoblastic leukaemias histological classification
acute lymphoblastic leukaemias... histological classification
  • precursor B- and T- cell lymphoblastic leukaemia/lymphoblastic lymphoma
proliferation of macrophages histiocytosis
proliferation of macrophages, histiocytosis

A) reactive proliferation of macrophages

  • bone marrow, many causes (hemosiderosis, aiha, viral infections)
  • lysosomal storage diseases (m. Gaucher, Niemann-Pick...)
proliferation of macrophages histiocytosis37
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
proliferation of macrophages histiocytosis38
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
proliferation of macrophages histiocytosis39
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
  • osteolytic x osteoplastic
  • prostate, breast, stomach, lung cancer
bone marrow necrosis
bone marrow necrosis
  • ischemia:
    • vascular collaps in hypercellular marrow
    • metastatic obstruction
    • sickle cell disease, DIC...
  • symptoms: pain, fever, hematopoietic precursors in peripheral blood
  • transplantation: bone marrow, peripheral stem cells
  • autologous x allogenneous (relatives, non-relatives)
  • indications:
    • hematological: tumours, immunodeficiency, anemias, b.m. aplasia
    • non-hematological: tumour metastasis
  • bone marrow suppression → graft
  • hypocellularity → proliferation
  • immunosuppression!
  • GvHD acute x chronic:
    • skin, intestine, liver
bleeding disorders
Bleeding disorders
  • cause:
  • defect in the vessel wall
  • platelet deficiency or dysfunction
  • coagulation factors disorder
bleeding disorders vascular
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
  • Marfan´s syndrome
bleeding disorders vascular46
bleeding disorders...vascular

2) acquired

a) avitaminosis C, ↑ corticosteroids

  • cutaneous, intramuscular, mucosal bleeding

b) purpura Henoch-Schönlein

  • circulating IC → skin, kidney
bleeding disorders plateles
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)
bleeding disorders plateles48
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
bleeding disorders plateles49
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
bleeding disorders plateles50
bleeding disorders...plateles
  • idiopathic thrombocytopenic purpura (ITP)
    • autoimmune origin
    • destruction in the spleen → splenectomy
    • bm +/- increased megakaryopoiesis
bleeding disorders plateles51
bleeding disorders...plateles

2) platelet dysfunction

  • adhesion disorder (Bernard-Soulier, m. von Willebrand)
  • aggregation disorder (thrombasthenia Glanzmann)
  • secretion disorder: tromboxan A2 inhibition (aspirin)
bleeding disorders coagulation factors
bleeding disorders...coagulation factors

C) coagulation disorders

1) hereditary deficiencies

a) hemophilia A (classic hemophilia)

  • f VIII (severe = activity < 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
bleeding disorders coagulation factors53
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

lymphatic vessels
lymphatic vessels

A) lymphoedema

  • lymph is protein-rich → lymphostasis leads to fibroproduction, +/- infectious and ulcerative complications

1) hereditary = Milroy´s disease

  • valvular disorder

2) acquired lymphoedema

  • lymphoedema praecox
  • secondary lymphoedema: obstruction and lymphostasis (mlg, inflammatory changes)
lymphatic vessels56
lymphatic vessels

B) lymphangiectasia

  • focal extension of lymphatic vessels
  • skin, small intestine (chylangiectasia)
  • → lymforhea (chylothorax...)
lymphatic vessels57
lymphatic vessels

C) lymphangiitis

  • lymph vessels draining the primary (infectious) focus
  • β hemolytic streptococci
  • + regional lymphadenitis
  • clinical: red subcutaneous line
  • histology:
    • lymphangiitis simplex
    • lymphangiitis purulenta: pus + fibrin → spreading → abscesses, trombophlebitis
lymfatic nodes structure
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
lymph nodes structure
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
lymph nodes regressive changes
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

lymph nodes inflammation
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
lymph nodes inflammation62
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!
lymph nodes inflammation63
lymph nodes...inflammation
  • progressive transformation of germinal centres
    • connection with HD (paragranuloma)
  • m. Castleman (angiofollicular hyperplasia)
    • „lolly pops“ follicles
    • unifocal bng x multifocal fatal
lymph nodes inflammation64
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
lymph nodes inflammation65
lymph nodes...inflammation
  • m. Rosai-Dorfman (masive sinusoidal histiocytoses)
    • intrasinusoidal macrophages with emperipolesis

d) mixed reactive hyperplasia

  • etio: toxoplasmosis (epitheloid granulomas)
lymph nodes inflammation66
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)
lymph nodes inflammation67
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
lymph nodes inflammation68
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
lymph nodes neoplasms70
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
lymph nodes neoplasms hd
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)
lymp nodes neoplasms nhl
lymp nodes...neoplasms...NHL

2) non Hodgkin lymphomas

  • predominance of neoplastic cells
  • elder patients
  • B and T cells
lymph nodes neoplasms b nhl
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, ENMZL=MALT, NMZL
  • hairy cell leukaemia (HCL)
  • diffuse large B-cell lymphoma (DLBCL)
  • Burkitt lymphoma
chronic lymphocytic leukaemia small lymphocytic lymphoma cll sll
chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL/SLL)
  • elderly patients
  • naive lymphocytes
  • indolent type of lymphoma
follicular lymphoma fcl
follicular lymphoma (FCL)
  • middle aged patients
  • centrocytes and centroblasts
  • indolent course but rellapsing!
mantle cell lymphoma mcl
mantle cell lymphoma (MCL)
  • „diffuse centrocytoma“
  • agressive type of lymphoma
  • t(11;14) – cyklin D1
marginal zone lymphoma mzl
marginal zone lymphoma (MZL)
  • mucosa associated lymphoid tissue (MALT) – GIT, bronchi…
  • association with chronic inflammation
diffuse large b cell lymphoma dlbcl
diffuse large B-cell lymphoma (DLBCL)
  • „waste basket“
  • transformation of small cell lymphomas
  • aggresive course x good reaction to therapy
burkitt lymphoma
Burkitt lymphoma
  • endemic x sporadic
  • younger patients
  • association with EBV infection
lymph nodes neoplasms b nhl80
lymph nodes...neoplasms...B-NHL
  • plasma cell neoplasms:
  • monoclonal gammopathy of undetermined significance (MGUS)
  • lymphoplasmacytic lymphoma (LPL), m. Waldenström
  • plasmacytoma
  • multiple myeloma
lymph nodes neoplasms t nhl
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
anaplastic large t cell lymphoma alcl
anaplastic large T cell lymphoma (ALCL)
  • young patients
  • typical translocation t(2;5)
mycosis fungoides sezary syndrom
mycosis fungoides/Sezary syndrom
  • primary skin lymphoma → generalisation = Sezary syndrom
  • structure:
    • white pulp: lymphoid tissue
    • red pulp: venous sinuses → hilus
  • splenomegaly: venosthasis, inflammation, neoplasms
  • hypersplenism = increased function → cytopenia
  • hyposplenism → susceptibility to certain bacterial infections
spleen regressive changes circulatory disorders
spleen...regressive changes, circulatory disorders

A) inborn anomalies

  • accessory spleens = spleniculi

B) regressive changes and circulatory disorders

1) amyloidosis

  • secondary (AA)
  • +/- hyposplenism
spleen regressive changes circulatory disorders87
spleen...regressive changes, circulatory disorders

2) storage diseases

3) hemolytic anemias

  • ↑ splenic function → splenomegaly
  • hereditary spherocytosis

4) chronic perisplenitis

5) splenic infarction

  • white (embolization, vasculitis)
  • red (thrombosis of lienal vein)
spleen regressive changes circulatory disorders88
spleen...regressive changes, circulatory disorders

6) chronic venosthasis

7) splenic rupture, bleeding

  • traumatic
spleen inflammation

A) inflammation

1) acute septic tumour

  • reaction to general infection x tumour lysis
  • clinically: tense capsula, soft tissue
  • histology: red pulp cellular, small abscesses (central pyemia)
spleen inflammation90

2) chronic inflammatory tumour

  • chronic infections (IE)
  • histology: red pulp hyperemia, reactive hyperplasia of the white pulp
  • malaria
  • TBC, histoplasmosis, leishmaniosis, trypanosomiasis
  • AIDS
spleen tumours

D) tumours and pseudotumours

1) cystic formations

  • posttraumatic pseudocysts
  • parasitary cysts

2) hamartoma (splenoma)

  • nodule
  • histology: chaotic sinuses a fibrous tissue = incorrect arrangement of the red pulp
  • +/- hypersplenism
spleen tumours92

3) hemangioma

  • histology: cavernous blood spaces, thrombosis

4) littoral cell angioma

  • phagocytosis → pancytopenia

5) inflammatory pseudotumour

  • histology: inflammatory cells + fibroproduction
spleen tumours93

6) malignant lymphomas

  • primary: SMZL
  • secondary:
  • more often
  • secondary infiltration by NHL, HD, CML, HCL

7) epithelial metastasis

  • microscopically
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
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
thymus dysgenesis

A) thymic dysgenesis

  • primary immunodeficiency syndromes (diGeorge, Nezelof...)
thymus regressive changes
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
thymus hyperplasia

C) thymic hyperplasia

  • primary hyperplasia
  • myasthenia gravis
  • histology: lymphoid hyperplasia, lymphatic follicles with germinal centres
  • Ab anti acetylcholin-receptors
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