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Spleen, LN, WBCs P. Babál UPA LFUK

Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

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Page 1: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Spleen, LN, WBCs

P. BabálUPA LFUK

Page 2: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Leucocytosis, Lymphocytosis

dif. dg.: reactive/neoplasticLeukocytosis – pathologicaly increased number of

granulocytes in peripheral blood (neutro-, eosino-, basophylic)

-neutrophylic

-bazophylic

-eosinophylic

Lymphocytosis (viruses)

Monocytosis

Plasmocytosis (rubella)

Leukemoid reaction, shift to the left (immature forms)

Page 3: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Leucopenia, Lymphocytopenia

Leukopenia – pathological decrease of granulocytes in peripheral blood (neutro-, eosino-, basophylic)

Lymphocytopenia – decrease

- Consequence of toxic effect, radiation, cytostatics...

Causes:

- immunologic (anti leukocyte antibodies, hapten type)

- toxic (some infect or medications – direct toxic effect)

- excessive destruction (spleen, paraneoplastic, AIDS)

- quality of leukocytes (ability of migration, phagocytosis...)

Page 4: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Lymphatic node

Page 5: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Lymphnodes

Reactive hperplasia: - follicles- cortex, paracortex- sinuses

Follicular hyperplasia: - RA, SLE, Sjögren syndrome- AIDS (...later loss of mantle zone)- Castleman disease: localized/multicentric - HHV8

Interfollicular hyperplasia – paracortex:- neoplastic- viral infects (EBV, measles, CMV,...- Phenytoin-induced (Ly, Eo, Pc, Ib)- SLE (Ib, Pc)

Sinus histiocytosis: - reactive (infect, trauma/surgery, tumor...)- Dermatopathic lymphadenopathy- Rosai-Dorfman disease- hemophagocytic sy. (infection, especially viral)

Page 6: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Infectious mononucleosis

HE

LMP1 (EBV)

Page 7: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Dermatopathic lymphadenopathy

Giemsa S100

Page 8: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Castleman disease

HE

CD 20

Page 9: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Lymphnodes

- primary/secondary neoplasms

- inflammatory processes

Lymphadenitisnonspecific/specific

Acute nonspecific lymphadenitis

- reaction to pathological process in the region(inflammation, neoplasm, degeneration...)

- LN is enlarged

- hyperplasia of B and/or T zone

- sinus histiocytosis

Page 10: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Lymphnodes

Lymphadenitis

Chronic nonspecific lymphadenitis

similar changes like in acute lymphadenitis

+ fibrosis

+ postinflammatory atrophy

+ histiocytic (dermatopathic, toxoplasma)

Purulent nonspecific lymphadenitis

Purulent intersticial inflammation

- abscessing/nonabscessing

Viral lymphadenitis

- transformation/stimulation of T or B lymphocytes

- blastic transformation of lymphocytes

Page 11: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Lymphnodes

Lymphadenitis

Specific lymphadenitis

- specific histopathologic finding

Tbc, BCG, sarcoidosis, syphilis, lepra

Etiologically specific lymphadenitis

- typical morphology...characteristic for etiological agens

Toxoplasmosis (Piringer-Kuchinka), EBV, cat-scratch, tularemia, yersinia...

Page 12: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Toxoplasmic lymphadenitis (Piringer-Kuchinka)

Page 13: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Cat-scratch disease

HE

Page 14: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Extranodal lymphatic tissue

MALT (mucosa associated lymphoid tissue)

- may react independently from other lymphatic system

- represents protective system of mucosas (GIT, respiratory system, conjunctiva, skin...

- both B and T lymphocytes, ...“homing“ – return to lamina propria of the mucosa after leaving (lymphatic vessels, ductus thoracicus, blood, MALT)

- extranodal lymphomas: Hodgkin/nonHodgkin

nonHodgkin: MALT lymphoma (indolent course)

low/high grade B or T lymphomas

Page 15: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Extranodal lymphatic tissue

MALT (mucosa associated lymphoid tissue)GITImmunoproliferative intestinal disease (IgA plasmocytes) – Mediteranian area

Enteropathy (gluten alergy) associated T-cell lymphoma

Waldayer ringB-cell lymphomas (+ extramedullary plasmocytoma)

NK/T lymphoma – nasal type

SkinT-lymphomas (Mycosis fungoides)

MALT (boreliosis), other B-lymphomas

Salivary, lacrimal, thyroid glandMALT, -> high grade B-lymphomas

MediastinumMALT (thymus)

Page 16: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

SpleenGalen: „organon pleunum mysterii“

Hyposplenism (asplenism)Atrophy - senile

- after diseases

Hypersplenism - splenomegalyHyperplasia - red pulp (phagocytic activity, extramedullary

hemopoesis, venostasis, storage diseases...)

- acute septic tumor of the spleen

- white pulp (immune reaction)

- extramedullary hemopoesis (PMF)

Hemolytic anemias

- spherocytosis, thalassemia, sicle cell disease..

- immunologic anemias, malaria

- thrombocytopenias

Page 17: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Spleen

Hypersplenism - splenomegaly

Primary tumors - B-cell lymphomas:

• Splenic marginal zone lymphoma →

• Hairy cell leukemia

• Hodgkin lymphoma

Secondary tumors

• CML, AML, Lymphomas (CLL)

• Carcinomas

Splenectomy - consequences: postsplenectomic infections

Page 18: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

ITP

Page 19: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Extramedullary hemopoesisin PMF

Page 20: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Spleen – other diseases

Inborn immunodeficienciesB or T cell deficit

Cysts- Epithelial cysts

- Parasitic cysts

- Posttraumatic cysts

Rupture of spleen- In disease: malaria, salmonelosis, tumor infiltration...

- Trauma (...implantation metastases = peritoneal splenosis)

Page 21: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Rupture of the spleen

Page 22: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Normal thymus

Page 23: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Thymus

Hypoplasia/agenesis

- inborn defects (DiGeorge syndrome, reticular dysgenesis)

- acquired (involution in young) – consequence of stress, malnutrition, radiation, corticoids...

Acute thymic involution

HE AE1 - cytokeratins

Page 24: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Inborn hypo/aplasia thymic cortex

Page 25: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Thymus

Hyperplasia

- follicular hyperplasia (B-cells)

Myastenia gravis, SLE, sclerodermia, Graves disease...

CD 20

Page 26: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Thymus

Thymoma

- Thymoma A: thymic epithelium + T lymphocytes

- Thymoma B: thymic epithelium (infiltration, local metastases)

- Thymic carcinoma, Thymic carcinoid (NET),

Lymphomas (B,T, cHL)

Page 27: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Thymoma type A

Page 28: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Thymoma type B

Page 29: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic

Thymic carcinoma

Page 30: Spleen, LN, WBCs - zona.fmed.uniba.sk · - Rosai-Dorfman disease - hemophagocytic sy. (infection, especially viral) ... - spherocytosis, thalassemia, sicle cell disease.. - immunologic