Malignant lymphoma

From WikiLectures

Lymphatic nodes with lymphoma.
Malignant lymphoma

Malignant lymphoma = tumor of lymphatic tissue (lymphatic nodes + extranodulary lymphatic tissue).

  1. B-lymphoma (80 %, usually more severe).
  2. T-lymphoma.
  3. NK-lymphoma.[1]

Units defined by their morphology + immunophenotypic features (CD Ag), cytogenetic traits, some even molecular-biological traits ([gene] translocations + protein production).[1]

Most important lymphomas

All malignant lymphomas can present so-called B symptoms: [1]

  • weight loss (10% / half a year),
  • subfebrile / febrile,
  • night sweats.


  • 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[edit | edit source]

  • 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[edit | edit source]

  • 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[edit | edit source]

T-cell lymphoblastic lymphoma[edit | edit source]
  • 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[edit | edit source]
  • mostly localized disease on the skin, scalp, bone and peripheral lymph nodes
Sporadic Burkitt lymphoma[edit | edit source]
  • 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[edit | edit source]
  • affects peripheral lymph nodes, mediastinum, kidneys, pericardium and lungs
  • manifestation of superior vena cava syndrome
Anaplastic large cell lymphoma[edit | edit source]
  • fever, weight loss
  • involvement of peripheral lymph nodes, mediastinum
  • skin, soft tissue and bone involvement

Diagnostics[edit | edit source]

  • 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[edit | edit source]

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

Therapy[edit | edit source]

  • 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[edit | edit source]

  • 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[edit | edit source]
  • 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[edit | edit source]
  • lymph node biopsy + histology
  • CT of the chest, CT/MR of the abdomen, PET
  • staging: Ann Arbor classification
Therapy[edit | edit source]
  • combined CHTR and RTR of affected areas
  • cytostatics used: cyclophosphamide, vincristine, corticosteroids, anthracyclines

References[edit | edit source]

Related articles[edit | edit source]

References[edit | edit source]

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