THE LYMPHOMAS

Divisi Hematologi-Onkologi DEPARTEMEN I.KESEHATAN ANAK FK-USU

Lymphoma : malignant proliferation of lymphoid cells arising lymphoid tissues ( lymph node , thymus , spleen )

Leukemia : malignancy arising from the bone marrow, which may include lymphoid cells.
Lymphoproliferative disorders (LPDs): polyclonal,nonmalignant accumulations of lymphocytes that occur when the immune system fails to control virally transformed lymphocytes.

Lymphomas of childhood The Malignant lymphomas: 1. Hodgkin’s disease 2. Non-Hodgkin’s lymphoma (NHL)

Male : female = 3 : 1 NHL : HD = 1..5 : 1 .. The malignant lymphoma    Approximately 10% of all cancer in children < 15 yr.. the third most frequently after leukemia and brain tumors.

Hodgkin’S Disease Insiden 0 – 15 tahun Male predominance Children have a better response to treatment Survival rate > 20 years  for 75% .

are not fixed to surrounding tissue Lymphadenopathy increasing in size after 2weeks or failing to resolve within 4-8 weeks. . discrete or matted together .Symptoms and signs      Painless cervical adenopathy (70-80%) Supraclavicular adenopathy (25%) Mediastinal mass Node > 2 cm.Ruberry texture.

pruritus .…symptoms and signs    Fatigue . weight loss . night sweats . cough Half of patients : asymptomatic mediastinal disease (adenopathy or anterior mediastinal mass) Splenomegaly or hepatomegaly : associated advanced disease . fever . anorexia.

measles. 5.CMV.rubella.renal and liver function Ann Arbor Staging . 6.chicken pox. 3. 2. Biopsy Imaging Laparotomy Bone marrow aspirates and trephines Blood tests:CBC. 4.ESR.viral titers for EBV.Diagnostic investigations and staging 1.

Ann Arbor staging system for Hodgkin’s disease Stage I : involvement of 1 lymph node region(1) or a single extralymphatic organ or site (IE) Stage II :involvement of 2 or more lymph node regions on the same side of the diaphragma (II) or solitary involvement of an extralymphatic organ or site and of 1 or more lymph node regions on the same site of diaphragma (IIE) Stage III : involvement of lymph node regions on both sides of the diaphragm (III) which may be accompanied by localized involvement of extra-lymphatic organ or site (IIIE) or by involvement of the spleen (IIIS) or both of the these (IIISE) Stage IV: Diffuse or disseminated involvement of 1 or more extralymphatic organs or tissues with or without associated lymph node enlargement .

Ann Arbor Staging .

Erythrocyte sedimentation rate. renal and liver function studies Radiologic studies ..RECOMMENDED STAGING PROCEDURES IN CHILDREN WITH HODGKIN’S DISEASE All patients     Hystory and physical examination Lymph node biopsy Laboratorium studies: CBC.platelet count.

3. 2. Bone marrow biopsy Exploratory laparotomy with splenectomy. 4.Selected indications 1. 5.retroperitoneal lymph node biopsies. 6.liver biopsy Oophoropexy Bone scan CT head and necksuspicion of waldeyer’s ring involvement Gallium scan .

3.Treatment 1. 2. Radiotherapy Chemotherapy Combined modality .

Cardiopulmonary dysfunction 3. Soft tissue and bone growth abnormalities 5. Sterility 4. Opportunistic and viral infections . Chemical or clinical hypothyroidism 2.Complications of therapy of Hodgkin’s Disease Minor treatment complications 1. Long term alteration of immune function Serious treatment complications 1. Psychosocial problems 2.

lymphoma..Complication of therapy of hodgkin’s Disease Potentially fatal complications 1.. Second malignant neoplasmas:leukemia.solid tumor Overwhelming bacterial septicaemia/meningitis postsplenectomy or postsplenic irradiation . 2.

Late recurrence Second tumors Growth impairment Endocrine dysfuntion Fertility Educational and psychologic functioning Organ dysfunction . 3. 2. 5. 4.Long-term sequelae of lymphoma therapy 1. 7. 6.

Childhood Lymphoma are rapidly proliferating.small non-cleaved cell lymphoma (Burkit lymphoma (BFM) . diffus malignancy Hystologically classified:lymphoblastic lymphoma (LL) . 5 – 15% of malignancy in children < 15 yr. high grade .NON – HODGKIN’S LYMPHOMA    Divers group of cancers .

Large Bcell lymphoma (LBCL) . Burkitt-like lymphoma (BLL) . Anaplastic large cell lymphoma ( ALCL) .

lymph nodes.  . liver . CNS. skin.Symptoms and signs  Childhood NHL: any site of lymphoid tissue. Extralymphatic sites : bone. testes. bone marrow. thymus. spleen.

epidural space . bone.skin.Location of tumor     Intra abdominal primary tumors : 31% Mediastinal : 27% Head and neck primary sites : 29% Tissue of Waldeyer’s ring Cervical lymph nodes Peripheral lymph nodes.

Lymphoma Sites .

rapidly enlarging lymphadenopathy Constitutional symptoms : fever.Initial symptoms       Cough Sore throat Abdominal pain Vomiting Adenopathy Painless. more suggestive of Hodgkin’s Disease .weight loss uncommon in NHL .night sweats .

orthopnea) Superior v. facial plethora.dyspnea.venous engorgement) result of mediastinal NHL .cava obstruction ( facial edema.These symptoms are a true emergencyrapid diagnosis and treatment .   Most rapidly growing human tumors Airways compression (cough.

  Pleural or pericardial effusions CNS and bone marrow involvement  Bone marrow contains more than 25% lymphoblast- patients are diagnosed with ALL .

a right lower quadrant mass. Abdominal pain. weight loss. irritability Bone pain  metastatic disease   . intussusception a child > 5 y Many children present: fever. distention.

cytogenetics Bone marrow .immunophenotyping.peripheral blood smear.electrolyte Biopsi: histopathologic . .Diagnostic     Physical examination CBC.renal and liver fuction.

groin.) or tumor outside of the abdomen or mediastinum (middle chest)  Stage 2: NHL is limited to one tumor with local lymph node involvement. or NHL is limited to a primary tumor of the gastrointestinal tract with/without involvement of local lymph nodes. or any primary NHL tumor within the thorax (trunk) or extensive NHL within the abdomen.. underarm.Jude Children ‘s Research Hospital Staging System for NonHodgkin’s Lymphoma  Stage 1: NHL is limited to one lymph node group (e. Stage 3: NHL includes tumors or lymph node groups on both sides of the diaphragm. By contrast. with/without other sites of involvement.g. lymphoblastic lymphoma that produces more than 25% malignant cells in the bone marrow is defined as leukemia. neck. or any NHL around the spine or the outermost membrane of the brain and spinal cord (dura mater). or NHL is limited to two or more tumors or lymph node groups on the same side of the diaphragm.St. etc. Bone marrow NHL is defined as 5% malignant cells in an otherwise normal bone marrow with normal blood counts and smears. Stage 4: NHL is in the bone marrow or central nervous system (CNS).   .

Lymphadenopathy  Enlargement of lymph nodes results from antigen-stimulated proliferation of lymphocytes and other cells intrinsic to the lymph node or from infiltration of nodes by extrinsic cells such as polymorphonuclear leukocytes or metastatic malignant cells .

HIV .stap hylococcus . syphillis 2.Viral :Epstein-Barr virus.Bacterial: Streptococcus. Fungal: histoplasmosis.Protozoa: Toxoplasmosis.cytomegaloviru s. Malaria .tularemia. Coccidioidomycosis 4.Infection 1. Rubella 3.br ucellocis.Causes of lymphadenopathy 1.mycobacteriosis.

2.Autoimmune Disease Rheumatoid arthritis.Storage Disease Niemann-Pick and Gaucher’s Disease . serum sickness. Autoimmune hemolytic anemia 3.SLE.

Malignancy Lymphoma .Metastatic Rhabdomyosarcoma . Malignant histiocytosis.4. sinus histiocytosis with massive lymphadenopathy . Thyroid carcinoma Histiocytosis : LCH . Leukemia.Drug reaction Phenytoin and others 5. Neuroblastoma.

Miscellaneous  Sarcoidosis  Kawasaki syndrome  Cat-scratch fever .6.

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