lymphoma | Anatomy2Medicine
Lymphoma Causes

lymphoma

 

  • Hodgkin lymphoma (Hodgkin disease)

 

      • a malignant neoplasm
      • Clinical features (e.g., fever, inflammatory cell infiltrates) resemble an inflammatory disorder.
      • characteristically affects young adults (predominantly young men)
      • an exception is nodular sclerosis, which frequently affects young women. (MCQ)
      • Associated manifestations often include

 

  • pruritus, fever, diaphoresis

 

        • leukocytosis reminiscent of an acute infection.
      • characterized in all forms by the presence of Reed-Sternberg cells. (MCQ)
      • The diagnosis of Hodgkin lymphoma depends on histologic finding of Reed-Sternberg cells. (MCQ)
      • Reed-Sternberg cells

 

  • binucleated, or multinucleated, giant cells (MCQ)

 

        • with eosinophilic inclusion-like nucleoli that may be the actual malignant cells of Hodgkin lymphoma.
        • Differing numbers are found in varying forms of Hodgkin lymphoma
        • severity of the disease variants is directly proportional to the number of Reed-Sternberg cells found in the lesions.
        • the greater the number of reactive lymphocytes in the Hodgkin lymphoma variant, the better the prognosis. (MCQ)

 

  • WHO classification of Hodgkin lymphoma
  • Lymphocyte-predominance Hodgkin lymphoma

 

          • large numbers of lymphocytes and histiocytes
          • a paucity of Reed-Sternberg cells
          • no association with EBV infection. (MCQ)
          • Peak incidence occurs in young and middle-aged men.

 

  • The prognosis is relatively good. (MCQ)
  • Lymphocyte-rich Hodgkin lymphoma

 

          • There is an association with EBV infection in 40% of cases
          • This variant is more common in men than in women. (MCQ)
          • The clinical course is moderately aggressive.

 

  • Mixed cellularity Hodgkin lymphoma

 

          • This variant is the one found most often in older persons
          • It is more common in men than in women.
          • Characteristic features include a
            • polymorphic infiltrate of eosinophils, plasma cells, histiocytes, and Reed-Sternberg cells,
            • areas of necrosis and fibrosis. (MCQ)
          • There is an association with EBV infection in 70% of cases. (MCQ)
          • The clinical course is moderately aggressive.

 

  • Lymphocyte depletion Hodgkin lymphoma

 

          • This variant is the least frequently occurring form of Hodgkin lymphoma.
          • Few lymphocytes, numerous Reed-Sternberg cells, and extensive necrosis and fibrosis are apparent.
          • There is an association with EBV infection in the great majority of cases

 

  • this variant is also more common in persons with human immunodeficiency virus infection. (MCQ)

 

          • has the poorest prognosis among all the variants. (MCQ)
        • Nodular sclerosis Hodgkin lymphoma
          • most frequently occurring form of Hodgkin lymphoma(MCQ)

 

  • Unlike other forms of Hodgkin lymphoma, this variant occurs more frequently in young women. (MCQ)

 

          • Nodular division of affected lymph nodes by fibrous bands

 

  • presence of lacunar cells, Reed-Sternberg cell variants, are characteristic. (MCQ)
  • The neoplasm often arises in the upper mediastinum or lower cervical or supraclavicular nodes.

 

          • There is rarely an association with EBV infection. (MCQ)

 

  • The prognosis is relatively good. (MCQ)
  • Clinical staging: Ann Arbor classification

 

        • This system of classification is based on the

 

  • degree of dissemination
  • involvement of extralymphatic sites
  • presence or absence of systemic signs such as fever

 

        • It is an essential part of the diagnostic evaluation of patients with Hodgkin lymphoma.
        • Although grading of histopathologic variants roughly correlates with clinical behavior, prognosis is better predicted by staging (Ann Arbor classification). (MCQ)

 

  • Ann Arbor Classification of Hodgkin and Non-Hodgkin Lymphomas(MCQ)

 

          • Stage I
            • Involvement of a single lymph node region (I)
            • involvement of a single extralymphatic organ or site (IE)
          • Stage II
            • Involvement of two or more lymph node regions on the same side of the diaphragm alone (II)
            • with involvement of limited contiguous extralymphatic organ or tissue (IIE)
          • Stage III
            • Involvement of lymph node regions on both sides of the diaphragm (III)
            • which may include the spleen (IIIS),
            • limited contiguous extralymphatic organ or site (IIIE), or both (IIIES)
          • Stage IV
            • Multiple or disseminated foci of involvement of one or more extralymphatic organs with or without lymphatic Involvement
          • Stages are further designated on the basis of absence (A) or presence (B) of systemic symptom (MCQ)

 

  • Non-Hodgkin lymphomas.
  • malignant neoplasms

 

      • arise from lymphoid cells or other cells native to lymphoid tissue
      • They originate most frequently within lymph nodes or in other lymphoid areas.
      • Tumor involvement of the periaortic lymph nodes is frequent.

 

  • WHO classification of B-Cell Neoplasms
  • Precursor B-cell neoplasm

 

          • Precursor B lymphoblastic leukemia/lymphoma (precursor B-cell acute lymphoblastic leukemia)

 

  • Mature (peripheral) B-cell neoplasms

 

          • B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma Lymphoplasmacytic lymphoma
          • Hairy cell leukemia
          • Plasma cell myeloma/plasmacytoma
          • Extranodal marginal zone B-cell lymphoma of MALT type Follicular lymphoma
          • Mantle cell lymphoma
          • Diffuse large B-cell lymphoma
          • Burkitt lymphoma/Burkitt cell leukemia
      • WHO classification of T-Cell Neoplasms

 

  • Precursor T-cell neoplasm

 

          • Precursor T lymphoblastic lymphoma/leukemia (precursor T-cell acute lymphoblastic leukemia)

 

  • Mature (peripheral) T-cell neoplasms

 

        • T-cell granular lymphocytic leukemia

 

  • Adult T-cell lymphoma/leukemia (HTLV1+)
  • Enteropathy-type T-cell lymphoma
  • Mycosis fungoides (Sézary syndrome)

 

      • Small lymphocytic lymphoma
        • a B-cell lymphoma
        • follows an indolent course
        • occurs most often in older persons
        • Diffuse effacement of lymph node architecture by small mature-appearing lymphocytes is characteristic. (MCQ)
        • widespread nodal involvement and involvement of the liver, spleen, and bone marrow frequently occur.
        • There is a close relationship to CLL(MCQ)
        • In the WHO classification, this disorder is called B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma.
        • The neoplastic cells express (MCQ)
          • surface immunoglobulin
          • pan–B-cell markers (e.g., CD19 and CD20)
          • positive for CD5 but negative for CD10.
      • Follicular lymphoma is a B-cell lymphoma,
        • often follow an indolent course in older persons
        • It is the most common form of non-Hodgkin lymphoma
        • Proliferation of angulated grooved cells that closely resemble the cells of the lymphoid follicular center, (MCQ)
        • commonly in a follicular (nodular) pattern is characteristic.
        • These cells express

 

  • surface immunoglobulin

 

          • B cell markers such as CD19 and CD20
          • sometimes positive for CD10, and are CD5 negative.
        • A cytogenetic change, t(14;18) is also characteristic. (MCQ)

 

  • Expression of bcl-2, an oncogene also occurs (MCQ)

 

        • bcl-2 codes for a mitochondrial protein that inhibits apoptosis. (MCQ)
      • Mantle cell lymphoma
        • arises from the mantle zone of lymphoid follicles
        • It is morphologically and immunophenotypically similar to small lymphocytic lymphoma, with slightly different cellular detail.

 

  • A translocation, t(11;14) is seen (MCQ)

 

          • t(11;14) results in activation of the cyclin D1 gene (bcl-1), is characteristic. (MCQ)
        • This disorder is most often manifest as a disseminated, aggressive, incurable disease
        • it occurs predominantly in older men.
      • Extranodal marginal zone B-cell lymphoma of MALT type
        • tends to arise in
          • sites of chronic inflammation as stomach in Helicobacter pylori gastritis. (MCQ)
          • sites of autoimmune disease such as the salivary glands in Sjögren syndrome, the thyroid in Hashimoto thyroiditis(MCQ)
        • It is often referred to as a MALToma (MALT = mucosa-associated lymphoid tissue).
      • Diffuse large B-cell lymphoma

 

  • usually presents as a large, often extranodal mass followed by widespread aggressive dissemination.

 

        • Leukemic involvement is rare
        • The disease most commonly occurs in older persons
        • however, the age range is wide and many of these lymphomas occur in children.

 

  • Precursor T lymphoblastic lymphoma/leukemia (precursor T-cell acute lymphoblastic leukemia)
  • often presents clinically as a combination of T-ALL and a mediastinal mass. (MCQ)
  • The disease occurs most often in children.

 

        • Convoluted-appearing nuclei are characteristic. (MCQ)

 

  • The lymphoma most often arises from thymic lymphocytes.
  • The disease rapidly disseminates and progresses to T-ALL. (MCQ)

 

      • Burkitt lymphoma

 

  • an aggressive B-cell lymphoma.

 

        • African form frequently involves the maxilla or mandible
        • American form usually involves abdominal organs.
        • There is a close linkage to EBV infection (especially in the African variety).
        • Histologic characteristics include a “starry-sky” appearance (MCQ)
          • As a result of rapid cell turnover, the lesions contain abundant cellular debris that is taken up by non-neoplastic macrophages, resulting in this appearance.
        • There is a close relationship to B-ALL (acute lymphoblastic leukemia of late-stage B-cell origin), which is called Burkitt cell leukemia in the WHO classification.
        • associated with a characteristic cytogenic change, t(8;14). (MCQ)
          • In this translocation, the c-myc proto-oncogene located on chromosome 8 is transposed to a site adjacent to the immunoglobulin heavy-chain locus on chromosome 14.
          • Increased expression of the c-myc gene, presumably caused by the proximity of regulatory sequences of the immunoglobulin heavy chain gene, is characteristic.

 

  • Mycosis fungoides

 

        • Cutaneous T-cell lymphoma(MCQ)
        • Presenting features include
          • erythematous, eczematoid, or psoriasiform process
          • progress to raised plaques, and then to a tumor stage.
        • Histologic characteristics include

 

  • dermal infiltrates of atypical CD4+ T cells with cerebriform nuclei. (MCQ)
  • Small pockets of tumor cells within the epidermis are referred to as Pautrier microabscesses. (MCQ)

 

        • The disease eventually disseminates to lymph nodes and internal organs.
        • Sézary syndrome.
          • leukemic form of cutaneous T-cell lymphoma (MCQ)

 

  • characterized by the combination of skin lesions and circulating neoplastic cells with cerebriform nuclei. (MCQ)