Patologi Sistem Retikulo Endhotelial_1

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Transcript of Patologi Sistem Retikulo Endhotelial_1

Prof. DR. Ambar Mudigdo, Sp.PA(K)

dr. Erna Sulistyowati, M.Kes

Lymph Node

Lymph Node1. AFF LYMPH. VES.

2. CAPSULE

3. PERI PHER SINUS

4. PARA FOLICULAR CORTEX/ T CELL ZONE

5. LYMPHOID FOLICLE/ B CELL ZONE

6. EFF LYMPH VES

7. ARTERI & VEIN

8. MEDULLA

9. MEDULLARY SINUSES

White cell Disorders

Defisiensi (leukopenia) Proliferatif

reaktif →infeksi neoplasma

Reactive leukocytosis Peningkatan leukosit Penyebab → mikrobial

→ non mikrobial

Reactive leukocytosis

Causes of leucocytosis Neutrophilic → acut bacterial infections Eosinophilic → allergic Basophilic → jarang,myeloproliferative

disease Monocytosis → chronic infections(TB),bact

endocarditis, rickettsiosis,malaria Lymphocytosis → accompanies with

monocytosis in chronic immunologic stimulation (TB,brucellosis); viral (hep A, CMV,EBV); Bordetella pertusis.

Infectious Mononucleosis Acute Self-limited disease Adolescent and young adult “Kissing disease” Caused by B lymphocytotropic EBV

(member herpesvirus family) Gx:

fever,sore throat and generalized lymphadenitis

Increased lymphocytes in blood (atypical) Humoral antibody response to EBV

Polyclonal activation and proliferation of B cells

Morphology Lymphocytosis (12.000-18.000/L) Atypical lymphocytes (abundant

cytoplasm,multiple nuclear vacuolation, oval, folded nucleus)

Lymph nodes: typically discrete,enlarged,post cervical,axillary and groin regions)

Spleen: enlarged Liver: impaired, atypical lymphocytes in

the portal areas and sinusoids

Infectious Mononucleosis

Infectious Mononucleosis

Atypical lymphocytes

Reactive Lymphadenitis

Infections and nonmicrobial inflammatory stimuli cause leucocytosis but also involve lymph nodes (defensive barriers)

Acute nonspecific lymphadenitis Chronic nonspecific lymphadenitis Chronic specific lymphadenitis

ACUTE NON SP LYMPHADENITIS

REACTIVE CHANGES : Micro organism Cell debris Foreign

material

cervicalaxillariesinguinal

Macros : Enlarged, swollen, grey-red, pain.

Micros : Lymphoid foll prominent, large germinal center,histiosit, neutrophil.

Reactive changes Cervical, axillaries, inguinal, mesentericMorphology : enlarged, swollen, grey-

red, distended caps tender.Histopathology : Lymph. Follicles

>>, Large germ C with mitotic activity, neutrophil infiltration. Sinuses cell hyper trophi / plasia.

ACUTE NON SP LYMPHADENITIS

Inguinal, axillaries, not tender. Histopathology:

follicular hyperplasia-B celldark zone, light zone, plasma cell, histiosit, PMN, eosinophyl.

Three patterns: Follicular hyperplasia Paracortical lymphoid hyperplasia Sinus histiocytosis

CHRONIC NON SP LYMPHADENITIS

CHRONIC NON SP LYMPHADENITIS

I. Follicular hyperplasiaactivated B cell, larged, round germinal center.

II. Paracortical lymphoid hyperplasiaReactive chages T cellViral Infection.

III. Sinus histiocytosis.Distention-prominent sinusoidLymph node draining cancer

CHRONIC SPECIFIC LYMPHADENITIS

TUBERCULOSA MACROS :

bergerombol, packed, central caseous.

MICROS :tubercle, cheese, necrotic, langhans cell,epitheloid cell.

CHRONIC SPECIFIC LYMPHADENITIS

Morphology : besar, multiple, bergerombol, tdk nyeri, livide, fistel

Histopatology: tuberc, epiteloid, caseosa, langhans.

Lokasi: Leher, Supraclav, axilla

NEOPLASTIC PROLIFERATIONS OF WHITE CELLS

Divided into 3 categories: Lymphoid neoplasms (HL,NHL,lyphocytics

leukemias, plasma cells dyscrasias) Myeloid neoplasms (acute myelogenous

leukemia, chronic myeloproliferative disorder,myelodisplastic syndr)

Hystiocytic neoplasms → proliferations of hystiocytes

LYMPHOID NEOPLASMA LYMPHOID NEOPLASMA

HODGKIN’S LIMPHOMA NON HODGKIN’S LYMPHOMA

LYMPHOMA

Hodgkin Disease

Chain nodes-spread characteristic Giant cells, Reed-Stenberg cell

induced Rx lymph, histiocyt, granulocyt.

Nodular sclerosismixed cellularitylymph predominant lymphocyte-depleted

Hodgkin Disease

Hodgkin DiseaseHodgkin Disease

Hodgkin disease

100% kel limfe Tunggal menyebar 20-40 th(30-35 th) Jenis

Nodular sclerosis : 65% Mixed cellularity : 25% Limfositik predominan :10% Limfositik depletion

Clinical staging of HD

HD & NHL

HDHD NHLNHL

Local lymph .CER-Local lymph .CER-ME.ST-P.AME.ST-P.A

MULTIPHERIPHERALMULTIPHERIPHERAL

CONTIQUITYCONTIQUITY NONO

EXTRA NODAL EXTRA NODAL UNCOMMONUNCOMMON

COMMONCOMMON

WALDEYER & WALDEYER & MESENTERIC MESENTERIC INVOLVED RAREINVOLVED RARE

COMMONCOMMON

NODULAR SCLEROSIS

Most common 65-75%, ♀, Young

Prognosa BAIK, Kel limfe Leher Supraclavicular, Mediastinum. Nodular, Variant Reed Stenberg, Lacunar cell,

collagen band, divide lymphositis-nodule.

Reed-Stenberg cell

Microskopis : Reed-stenberg

cell: besar, binukleus, berhadap-kaca, kadang-kadang multi nuc, single lob, sekitar halo area

Lacunar cell : nodular sel

HD; mixed cell type

25%,♂, older, prognosa baik Diffuse heterogen cell infiltrat, classic RS

cell, some mononucleus v

HD; lymphocytes predominant

6%, ♂, <35 th, prognosa exelent Lymph leher, axilla Nodular inf by lymph small round,RS cell

jarang, popcorn cell, << necrosis, fibrosis

Non Hodgkin Lymphomas

B-cell lymphoma

Chronic lymphocitic lymphoma Folicular lymphoma Diffus large B cell lymphoma Burkitt lymphoma Plasma cell neoplasm, etc.

MULTIPLE MYELOMA

PLASMA CELL bone, ♂ > ♀ , Immunoglobulin > , prot Bence

Jones (urine), Midle age (50). Clinic :

Multiple bone destruct: bone pain; Vert, Ribs, Skull, pelvis, femur, Rö bulat oval, batas fract. Patol.Micros : plasma cell >> pleomorphic, exentric nucleus plasma blast spleen, liver, kidney, lungs

Recurrent inf St.aureus, Str.pneumoniae Renal insuff; prot Bence Jones

Folicular lymphoma

NHL : paling sering(45%) ♀~♂; dewasa Micros : nodular struktur, small cell irreg.

inti lebih besar, cell chrom >>, nucleoli >

Clinical : painless limfadenopati, general, extra nodal rare, median survival 7-9 th

Tx : Chemoteraphy, radiasi, palliative Tx

Diffuse Large B-cell Lymphoma

20% NHL ♀<♂, 60 th Micros :

Diffus patern Large cell : 4-5x limf kecil Nuclei : bulat-oval, cleaved-lobulasi Anak inti : 2-3 atau 1 di tengah

Clinic :pembesaran kel. limf., kadang-kadang extra nodal : Git, bone, brain, skin.

Prog : Jelek Tx : Intensive mix chemo Tx complete remission 60-

80%, 50% for several years.

T cell lymphoma & natural killer cell N

1. Peri T cell L UNSP2. Adult T cell Lymphoma3. Mycosis fungoides4. Hodgkin disease

Klasifikasi bervariasi mnrt : Asal :sel B,sel T. Struktur : FOL,DIF. Vol.sel : LARGE,SM. DIFERENSIASI : WORKING FROM.,REAL

CLAS.,RAPPAPORT.

Precursor B Cell & T cell NPrecursor B Cell & T cell N

Acut Lymphoblastic LAcut Lymphoblastic L

di AS : 2500 KASUS BARU/THdi AS : 2500 KASUS BARU/TH < 15 th, kulit putih 2X< 15 th, kulit putih 2X ♂ ♂ > atau = ♀, balita, < 15 th.> atau = ♀, balita, < 15 th. Mic : Str Norm kel lif rusak, sel limfoblas Mic : Str Norm kel lif rusak, sel limfoblas

dominan : Besar. Inti Lobulated, Mitosis dominan : Besar. Inti Lobulated, Mitosis >>>>

Clinn : Anemia, Lemah, Panas Clinn : Anemia, Lemah, Panas acut. acut. Infeksi, Bone pain, Spleen & liver Infeksi, Bone pain, Spleen & liver enlargement, General limfadenopati, dllenlargement, General limfadenopati, dll

ACUT LIMFOBLASTIC LIMFOMA/LEUKEMIAACUT LIMFOBLASTIC LIMFOMA/LEUKEMIA

Chronic lymphocitic / small cell limfoma

Sering pada dewasa Small B cell pd sirkulasi (leukemia) Micros : str NL rusak, Small limfosit

predominan, inti kecil, kombinasi dgan sel > besar (prolimfositic)

Clin : > 50 tahun ♀< atau = ♂, sering asimptom, BB↓, lemah,

anoreksi, Lekosit >>, Limfadenopatia, spleen+ Liver

Burkitt limfoma

Endemic-sporadic-agresif (Afrika). Mic : invasi intermed size sel

limfosit, inti oval-bulat, nucleoli>1 kromatin kasar, mitosis>> starry sky patern (macrophages, ingest nuc debris)

Clinic : extra nodal manifes, mandibula,

organ intra Abdomen. Children, muda.

SPLEEN

SPLEEN

150 GR : 12 x 7 X 3 CM MACROS : KAPSUL TIPIS, ABU-ABU

TRABEKULA PD. PARENKIM MERAH DGN BERCAK PUTIH-WHITE PULP. RED PULP. SINUSSOID DGN KAPILER DD TIPIS

FUNGSI

1. Pembentukan sel darah (diluar sumsum tulang).

2. Proses hemolisis3. Reservoir darah, mobilisasi bila perlu4. Reaksi pertahanan-kekebalan tubuh,

produksi limfosit dan zat anti

Gangguan sirkulasi

1. Bendungan akut2. Bendungan menahun3. Sindroma banti : splenomegali

kongestif >>, lekopeni, anemi.4. Infark : Obstr. Emboli cor single/

multiple-Baji. Gamna-Gandy bodies : bercak abu2-coklat

(fibrosis+ endapan pigmen) pd Banti sindrom

Splenomegali

1. Infeksi : Typ. TBC, Mal syph2. Congestiv : Cirh, decomp, portalthrom3. Limfo-hematogen dis.4. Immunogenic5. Storage dis : gaucher, niemann-Pick6. Lain : amyloid, neopl

Neoplasm : JarangNeoplasm : Jarang

Benign : Hemangioma, Limfangioma, fibromaBenign : Hemangioma, Limfangioma, fibroma