the hematopoietic and lymphoid systems l.
Download
Skip this Video
Loading SlideShow in 5 Seconds..
the hematopoietic and lymphoid systems PowerPoint Presentation
Download Presentation
the hematopoietic and lymphoid systems

Loading in 2 Seconds...

play fullscreen
1 / 100

the hematopoietic and lymphoid systems - PowerPoint PPT Presentation


  • 345 Views
  • Uploaded on

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

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about 'the hematopoietic and lymphoid systems' - doyle


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
hematopathology
hematopathology
  • blood
  • lymphoid organs
    • central:
      • bone marrow
      • thymus
    • peripheral:
      • lymph nodes
      • MALT (Waldeyer´s ring, intestine...)
      • splenic white pulp
hematopathology3
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
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
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
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
anemias..hemolytic..intrinsic

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
anemias..hemolytic..intrinsic
  • 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
anemias..hemolytic..intrinsic
  • acquired
  • membrane defect: paroxysmal nocturnal hemoglobinuria)
    • ↓resistance against C3
    • granulocytes and plateles affected too → hemolysis, +/- trombotic complications and ↑ susceptibility to infections
anemias hemolytic extrinsic
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
anemias..hemolytic..extrinsic
  • autoantibodies
    • idiopathic (primary), drug associated, SLE
    • Coombs tests
anemias hemolytic extrinsic17
anemias..hemolytic..extrinsic

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
leukopenia
leukopenia

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
slide30
CMPD

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
slide31
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
slide32
CMPD

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
metastasis
metastasis
  • 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
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
transplantation43
transplantation
  • 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
spleen85
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
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
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
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
spleen tumours
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
spleen...tumours

3) hemangioma

  • histology: cavernous blood spaces, thrombosis

4) littoral cell angioma

  • phagocytosis → pancytopenia

5) inflammatory pseudotumour

  • histology: inflammatory cells + fibroproduction
spleen tumours93
spleen...tumours

6) malignant lymphomas

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

7) epithelial metastasis

  • microscopically
thymus structure
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
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
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
thymus...hyperplasia

C) thymic hyperplasia

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