Lymphopoietic System-kbk Blok Hematologi
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Transcript of Lymphopoietic System-kbk Blok Hematologi
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DISORDERS OF
LYMPHOPOIETIC
SYSTEM
KBK SEMESTER IV
BLOK HEMATOLOGI
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LYMPHOPOIETIC SYSTEM :
Circulating B & T lymphocytes
Lymphoid organs : lymph node, spleen,
thymus, MALT
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Lymphocytes : all derived from Bone
Marrow stem cells.
Thymus : T cells BM : B cells
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T lymphocytes
Lymphocytic stem cells migrate to thymus
exposed with thymic hormones
surface receptor (CD 2) as recognize Ag,CD 2 present on membrane + CD 3
marker T cells CD 5, CD 4 (helper)
or CD 8 (suppressor) migrate to
lymph nodes, spleen and peripheralblood.
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B lymphocytes
Acquired repertoire cytoplasmic & cell
surface Ag in bone marrow.
The earliest B cell antigens are CD19,CALLA (common acute leukemia/
lymphoma antigen, CD10) on the cell
membrane, and the nuclear antigen TdT.
Mature genes for immunoglobulin
heavy chains are rearranged
synthesis of IgM molecules .
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In precursor B cells, IgM expressed in
cytoplasm. After activation and clonal
expansion in germinal centers, Blymphocytes migrate B cell-dependt
medullary cords of lymph nodes Ig-
secreting plasma cells or exit lymph
nodes as memory B lymphocytes.
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Mature B cells express surface pan B
cell antigens CD19, CD20, CD22, plus Ig
heavy and light chains.When activated by antigen and
stimulated by an appropriate T helper
cell, B cells develop into plasma cells
that synthesize and exportimmunoglobulins.
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Mature B cells express surface pan B
cell antigens CD19, CD20, CD22, plus Ig
heavy and light chains. When activatedby antigen and stimulated by an
appropriate T helper cell, B cells develop
into plasma cells that synthesize and
export immunoglobulins.
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NATURAL KILLER (NK) AND
CYTOTOXIC CELLSA small proportion of lymphocytes
express neither B nor T cell
differentiation antigens.Act as cytotoxic or natural killer cells (NK
cells.
CD56+, CD3-, and CD8-
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LYMPH NODES
Outer cortex and an inner medulla .
B-cell-dependent cortex :
Inactive follicles (primary follicles)
cohesiveaggregates of small, normal-appearing
lymphocytes.
Active follicles (secondary follicles) Germinal
centers contain large lymphocytes
(centroblasts) and small lymphocytes with
cleaved nuclei (centrocytes) & scattered
macrophages.
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Reactive Hyperplasia of Lymph
Nodes Response to Infections,
Inflammation or Tumors :Acute suppurative or necrotizing
lymphadenitis.
Follicular Hyperplasia : Hyperplasia ofsecondary follicles (germinal centers)
and plasmacytosis of medullary cords
indicate B lymphocyte immunoreactivity
nonspecific reactive follicularhyperplasia etiology not known (viral,
drug/inflammatory), AIDS.
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Interfollicular Hyperplasia Reactivenonspecific interfollicular hyperplasiaviral infections or immunologic reactions mononucleosis, varicella-herpeszoster infection, measles, andcytomegalovirus lymphadenitis, SLE.
Mixed Patterns hyperplasia toxoplasmosis, Cat-scratch disease,lymphogranuloma venereum andtularemia
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Sinus histiocytosis increase tissue
macrophages (histiocytes) of subcap-
sular and trabecular sinuses.
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MALIGNANT LYMPHOMA
Non Hodgkin Lymphoma.
Hodgkin Lymphoma.
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The NHLs are a heterogeneous group oflymphoproliferative malignancies with differing patterns ofbehavior and responses to treatment
Like Hodgkins lymphoma, NHL usually originates in
lymphoid tissues and can spread to other organs.
NHL, however, is much less predictable than Hodgkinslymphoma and has a far greater predilection todisseminate to extra nodal sites.
The prognosis depends on the histological type, stage,
and treatment.
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EPIDEMIOLOGY
4-5% of cancer in US
2005: 58 875 cases, 18 840 will die
Both adults & children Men are more common than women
White > african & asian american
Risk of getting NHL during lifetime :
- Men : 1/46- Women : 1/55
Risk of dying : 1/100
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RISK FACTOR
Men > Women
Age , Risk
Weakened Immuned system
- inherited disease
- autoimmune disease
- human immunodeficiency virus (HIV)
- drugs given because you had an organ
transplant.
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infected with
- HTLV-1
- EBV
- Helicobacter pylori exposed to certain chemicals
- ingredients in pesticides
- herbicides
- solvents- fertilizers
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SIGNS & SYMPTOMS
Most common : painless swelling of the lymph nodes in the neck,underarm (axilla), or groin.
Other :
- Unexplained fever
- Night sweats- Constant fatigue
- Unexplained weight loss and anorexia (poor appetite)
- Itchy skin (pruritus)
- Reddened patches on the skin
Symptoms above cant 100% determine NHL- flu, other infection
- only doctor can make diagnosis
http://en.wikipedia.org/wiki/Feverhttp://en.wikipedia.org/wiki/Night_sweatshttp://en.wikipedia.org/wiki/Fatiguehttp://en.wikipedia.org/wiki/Weight_losshttp://en.wikipedia.org/wiki/Anorexiahttp://en.wikipedia.org/wiki/Itchhttp://en.wikipedia.org/wiki/Itchhttp://en.wikipedia.org/wiki/Anorexiahttp://en.wikipedia.org/wiki/Weight_losshttp://en.wikipedia.org/wiki/Fatiguehttp://en.wikipedia.org/wiki/Night_sweatshttp://en.wikipedia.org/wiki/Fever -
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Swollen Lymph Node
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DIAGNOSIS Physical examination Medical history
Biopsy
- excisional
- incisional
- fine needle aspiration
Imaging studies
- chest x-ray
- computed tomography (CT) scan
- PET scan- MRI
- Lymphangiogram:
http://www.seattlecca.org/patientsandfamilies/adultCare/clinicalProgs/lymphoma/Definitions.htmhttp://www.seattlecca.org/patientsandfamilies/adultCare/clinicalProgs/lymphoma/Definitions.htmhttp://en.wikipedia.org/wiki/Lymphangiogramhttp://en.wikipedia.org/wiki/Lymphangiogramhttp://www.seattlecca.org/patientsandfamilies/adultCare/clinicalProgs/lymphoma/Definitions.htmhttp://www.seattlecca.org/patientsandfamilies/adultCare/clinicalProgs/lymphoma/Definitions.htm -
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To diagnose whether the cancer has spread
around the lymph system or to other areas.
- CBC- Blood chemistry analysis
- Lumbar puncture
- Bone marrow test
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Biopsy
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TREATMENT
Chemotherapy
Radiation Therapy
Bone Marrow Transplantation Biological Therapy
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Chemotherapy
Uses drugs to kill cancer cell
Enter bloodstream & travel through body
Disadvantages kill healthy cell
Drug combination in cycle Given alone or with radiation therapy
Vein or mouth
Side effect :
- Nausea and vomiting
- Fatigue or tiredness- Hair loss
- Esophagitis or irritation of the swallowing tube
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Radiation Therapy
External beam
Linear accelerator
Short burst of x-ray
Square-shaped manner
Also kill other cell
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Bone Marrow Transplantation
injection of healthy stem cells from a
donor's bone marrow into your vein
The new stem cells travel through thebloodstream to your bone cavities.
Stem cells are cells that can produce red
blood cells, white blood cells, and
platelets.
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Biological Therapy
using medications or substances made
by the body to increase or restore your
body's natural defenses against cancer It is also called immunotherapy
Monoclonal antibodies and interferon are
examples of biological therapy used to
treat some types of lymphoma.
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CLASSIFICATION
Morphologic
Immune-Based
The Working FormulationUpdated REAL / WHO
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Morphologic Classification
type of involvement
1956 Rappaport Classification
Simple, separate more aggressive & lessaggressive
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Immune-Based Classification
Luke & Collins (1974) US
Kiel Classification Europe
Fits theoretic concepts of development of
lymphoid cell
Not gained universal acceptance bcoz :
- their complexity
- need ancillary test to reach precise diagnoses
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Working Formulation
Classification
1975, NCI develop The Working
Formulation of Non-Hodgkins
Lymphoma for Clinical UsageCurrently used in US
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Working Formulation Rappaport Classification
Low-grade
A. Small lymphocytic, consistent with chronic
lymphocytic leukemia (SL)
B. Follicular, predominantly small-cleaved cell
(FSC)
C. Follicular, mixed small-cleaved and large
cell (FM)
Intermediate-gradeD. Follicular, predominantly large cell (FL)
E. Diffuse, small-cleaved cell (DSC)
F. Diffuse mixed, small and large cell (DM)
G. Diffuse, large cell, cleaved or noncleaved
cell (DL)
High-grade
H. Immunoblastic, large cell (IBL)
I. Lymphoblastic, convoluted or
nonconvoluted cell (LL)
J. Small noncleaved-cell, Burkitt's or
non-Burkitt's (SNC)
- Diffuse lymphocytic, well-differentiated (DLWD)
- Nodular lymphocytic,poorly differentiated (NLPD)
- Nodular mixed, lymphocytic and histiocytic (NM)
- Nodular histiocytic (NH)- Diffuse lymphocytic, poorly differentiated (DLDP)
- Diffuse mixed, lymphocytic and histiocytic (DM)
- Diffuse histiocytic (DH)
- Diffuse histiocytic (DH)
- Diffuse lymphoblastic (DL)
- Diffuse undifferentiated Burkitt's or non-Burkitt's
(DU)
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Updated REAL / WHO
Classification
Working formulation became outdated
Since 1995, members of the European and AmericanHematopathology societies have been collaborating on anew World Health Organization (WHO) classification,
which represents an updated version of the REALsystem.
3 major categories of lymphoid malignancies based onmorphology and cell lineage:
- B-cell neoplasms
- T-cell/natural killer (NK)-cell neoplasms
- Hodgkins lymphoma
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I. B-cell neoplasm
IA Precursor B-cell neoplasm:
precursor B-acute lymphoblast
leukemia/lymphoblast lymphoma
(B-ALL, LBL).
IB Peripheral B-cell neoplasms.
1. B-cell chronic lymphocyticleukemia/small lymphocytic lymphoma.
2. B-cell prolymphocytic leukemia.
3. Lymphoplasmacyticlymphoma/immunocytoma.
4. Mantle cell lymphoma.
5. Follicular lymphoma.
6. Extranodal marginal zone B-celllymphoma of MALT type.
7. Nodal marginal zone B-cell lymphoma (monocytoid B-cells).
8. Splenic marginal zone lymphoma (villous lymphocytes).
9. Hairy cell leukemia.
10. Plasmacytoma/plasma cell myeloma.
11. Diffuse large B-cell lymphoma.
12. Burkitt's lymphoma.
II. T-cell and putative NK-cell neoplasm
IIA.Precursor T-cell neoplasm: precursor
T-acute lymphoblast
leukemia/lymphoblast lymphoma
(T- ALL, LBL).
IIB. Peripheral T-cell and NK-cell
neoplasms.
1. T-cell chronic lymphocyticleukemia/prolymphocytic leukemia.
2. T-cell granular lymphocytic leukemia.
3. Mycosis fungoides/Szary syndrome.
4. Peripheral T-cell lymphoma, nototherwise characterized.
5. Hepatosplenic gamma/delta T-celllymphoma.
6. Subcutaneous panniculitis-like T-celllymphoma.
7. Angioimmunoblastic T-cell lymphoma.
8. Extranodal T-/NK-cell lymphoma, nasaltype.
9. Enteropathy-type intestinal T-celllymphoma.
10. Adult T-cell lymphoma/leukemia (HTLV1+).
11. Anaplastic large cell lymphoma, primarysystemic type.
12. Anaplastic large cell lymphoma, primarycutaneous type.
13. Aggressive NK-cell leukemia.
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Hodgkins lymphoma (Hodgkins disease)
A. Nodular lymphocyte-predominant Hodgkinslymphoma.
B. Classical Hodgkins lymphoma.
1. Nodular sclerosis Hodgkins lymphoma.
2. Lymphocyte-rich classical Hodgkins
lymphoma.
3. Mixed-cellularity Hodgkins lymphoma.
4. Lymphocyte-depleted Hodgkins
lymphoma
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9. PREVENTION
Avoid Repeated Exposure to Certain
Chemicals
Avoid Exposure to HIV