Malignant lymphoma

Lymphatic nodes with lymphoma.

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

Clinical picture

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

References

Related articles

• Hodgkin lymphoma
• Non-Hodgkin lymphoma
• CNS lymphoma
• Skin lymphoma

References

• DÍTĚ, P., et al. Vnitřní lékařství. 2. edition. Praha : Galén, 2007. ISBN 978-80-7262-496-6.