Malignant lymphoma

Malignant lymphoma = tumor of lymphatic tissue (lymphatic nodes + extranodulary lymphatic tissue).
 * Hodgkin lymphoma.
 * Non-Hodgkin lymphoma (NHL).
 * 1) B-lymphoma (80 %, usually more severe).
 * 2) T-lymphoma.
 * 3) NK-lymphoma.

Units defined by their morphology + immunophenotypic features (CD Ag), cytogenetic traits, some even molecular-biological traits ([gene] translocations + protein production).

Most important lymphomas
 * diffuse large B-cell lymphoma (30 %),
 * follicular lymphoma (22 %),
 * MALT-lymphoma (8 %),
 * chronic B-cell leukemia/lymphocytic lymphoma(7 %),
 * Mantle cell lymphoma (6 %).

All malignant lymphomas can present so-called B symptoms:
 * weight loss (10% / half a year),
 * subfebrile / febrile,
 * night sweats.

MALIGNANT LYMPHOMAS IN CHILDREN

 * 15% of malignant tumors in children and adolescents - the 3rd most common group of malignancies
 * incidence increases with age:
 * NHL more common in children under 10 years of age
 * HL 2x more often than NHL in adolescents older than 15 years

Non-Hodgkin lymphomas

 * a heterogeneous group of tumors of the lymphatic system
 * in children, mainly tumors with a high degree of malignancy
 * more often in boys

Etiology and pathogenesis

 * the formation of lymphoma as a result of genetic aberrations affecting lymphocyte proliferation, differentiation and apoptosis
 * higher incidence in children with primary and secondary immunodeficiencies
 * Burkitt's lymphoma - associated with EBV infection, endemic in equatorial Africa
 * sporadic Burkitt's lymphoma - children in Europe and America, unrelated to EBV infection, has different clinical manifestations

T-cell lymphoblastic lymphoma

 * dyspnea due to mediastinal tumor
 * taking the orthopneic position
 * Superior vena cava obstruction - symptoms of superior vena cava syndrome
 * the abdominal cavity is also often attacked - hepatosplenomegaly and kidney infiltration

Lymphoblastic lymphoma from B-precursors

 * mostly localized disease on the skin, scalp, bone and peripheral lymph nodes

Sporadic Burkitt lymphoma

 * involvement of the abdominal cavity
 * ileocecal intussusception in 1/4 of children
 * pain in P lower quadrant
 * dif.dg. appendicitis in these patients difficult
 * complete resection and end-to-end anastomosis
 * massive infiltration of the mesentery, retroperitoneum, peritoneum, kidneys, ovaries

Diff. large cell lymphoma

 * affects peripheral lymph nodes, mediastinum, kidneys, pericardium and lungs
 * manifestation of superior vena cava syndrome

Anaplastic large cell lymphoma

 * fever, weight loss
 * involvement of peripheral lymph nodes, mediastinum
 * skin, soft tissue and bone involvement

Diagnostics

 * cytomorfologie, histomorfologie, immunofenotypizace
 * biopsy of nodes or extranodal areas, pleural or peritoneal effusion puncture, bone marrow aspiration
 * respiratory patient with superior vena cava syndrome - !! very risky for an invasive diagnostic procedure - postponing the procedure, 24-48 hours of corticosteroid therapy, possibly in combination with cyclophosphamide
 * determining the extent of the disease - CT of the chest and abdomen, PET, examination of the cerebrospinal fluid to rule out meningeal infiltration

Differential diagnosis

 * Hodgkin's lymphoma
 * post-transplant lymphoproliferative disease
 * autoimmune lymphoproliferative disease

Therapy

 * children with lymphoblastic lymphomas - application of ALL therapy protocols
 * Burkitt's lymphoma, B-ALL, DLBCL - short and repeated blocks of intensive therapy (cyclophosphamide, MTX, vincristine)
 * complications of therapy:
 * urate nephropathy - trp. rasburicase (recombinant urate oxidase)
 * acute infection

Hodgkin lymphoma

 * the incidence is the same in both sexes
 * most patients have high titers of EBV antibodies - activation of EBV infection may precede the development of HL
 * division of HL:
 * classic - a tumor of lymphatic tissue from Hodgkin cells (mononuclear) and Reed-Sternberg multinuclear cells; 4 subtypes
 * HL with lymphocytic proliferation - from B-lymphocytes, with nodular proliferation of isolated large tumor cells

Clinical picture

 * painless swelling of the supraclavicular and cervical nodes
 * involvement of lymph nodes in the mediastinum
 * systemic manifestations
 * fatigue, loss of appetite
 * B-symptoms: fever, night sweats, weight loss of more than 10% in the 6 months before diagnosis
 * itchy skin (symptom of advanced disease)

Diagnostics

 * lymph node biopsy + histology
 * CT of the chest, CT/MR of the abdomen, PET


 * staging: Ann Arbor classification

Therapy

 * combined CHTR and RTR of affected areas
 * cytostatics used: cyclophosphamide, vincristine, corticosteroids, anthracyclines

Related articles

 * Hodgkin lymphoma
 * Non-Hodgkin lymphoma
 * CNS lymphoma
 * Skin lymphoma