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Primary Cutaneous Marginal Zone B-Cell Lymphoma

PCMZL


Presentation

  • Large cell transformation and head and neck presentation may be associated with a worse prognosis.[ncbi.nlm.nih.gov]
Fishing
  • FISH assay with a MALT1 dual color break apart translocation probe revealed the absence of MALT1 translocations in all cases.[ncbi.nlm.nih.gov]
  • The test is ultrasensitive and detects extremely low amounts of biomarkers remaining in blood, which can be missed by cytogenetic methods, such as FISH, karyotype, or flow cytometry.[dovemed.com]
  • FISH analysis of MALT lymphoma-specific translocations and aneuploidy in primary cutaneous marginal zone lymphoma. J Pathol 2005; 205 :302–310. 16. Gallardo F, Bellosillo B, Espinet B, et al.[nature.com]
  • As part of the diagnostic process, our specially trained dermatopathologist performs wide-ranging, leading-edge molecular testing for the most accurate diagnosis, including FISH analysis (fluorescent in situ hybridization) Bone marrow aspiration and biopsy[cityofhope.org]
Heat Intolerance
  • intolerance, recurrent rashes, abdominal pain, and a general feeling of illness If the brain is involved, then neurological symptoms such as the following may be observed: Confusion Tinnitus (ringing in the ears) Hearing and visual impairment If the[dovemed.com]
Tinnitus
  • Associated autoimmune disorders, which can cause joint and muscle pain, heat intolerance, recurrent rashes, abdominal pain, and a general feeling of illness If the brain is involved, then neurological symptoms such as the following may be observed: Confusion Tinnitus[dovemed.com]
Visual Impairment
  • impairment If the gastrointestinal (GI) tract is involved, then GI tract symptoms such as the following may be observed: Ulcers Diarrhea Inflammation GI bleeding Liver and spleen enlargement Joint inflammation and fluid accumulation (edema) can occur[dovemed.com]
Subcutaneous Nodule
  • This 46-year-old woman presented with a subcutaneous nodule on her right forearm. With the combined morphology,the immunophenotype, and molecular analysis, we diagnosed this lesion as PCMZL.[ncbi.nlm.nih.gov]
  • nodules. [1] : 741 Lymphomas included in this group are: [1] : 741 [2] Primary cutaneous immunocytoma Marginal zone B-cell lymphoma Mucosa-associated lymphoid tissue lymphoma See also [ edit ] Cutaneous B-cell lymphoma Skin lesion References [ edit ][en.wikipedia.org]
Disfiguring Scar
  • These therapies may not always be indicated in young patients or in patients with pCBCL located in the face, where surgery or radiotherapy may leave disfiguring scars or radioderma.[ncbi.nlm.nih.gov]
Subcutaneous Mass
  • Case 2, a 68-year-old Chinese male, had a subcutaneous mass next to back of the left ear with a size of 9.0 cmx5.0 cm, and he underwent a operation one year previously because of subcutaneous mass in the same site.[ncbi.nlm.nih.gov]
Facial Numbness
  • numbness If the abdomen is affected, it can cause intestinal obstruction that results in urine outflow obstruction and kidney damage Blood loss may occur due to frequent or heavy bleeding, giving rise to severe anemia The leukemic cells can overgrow[dovemed.com]
Kidney Failure
  • Hence, measures to protect the individual’s fertility must be considered, before starting chemotherapy Tumor lysis syndrome: This can occur due to chemotherapy treatment and can result in kidney failure, if not recognized promptly How is Primary Cutaneous[dovemed.com]
Meningism
  • Due to this, individuals are more vulnerable to infections; there is an increased risk of developing serious complications from such infections If the condition spreads to the brain and central nervous system, it can give rise to: Inflammation of the meninges[dovemed.com]

Workup

  • Follow-up Patients should be seen in an outpatient setting at least every 6 months for a clinical workup. They do not require any additional laboratory workup unless enlargement of the regional lymph nodes has occurred.[emedicine.medscape.com]
  • A systematic review Workup for Nodal Marginal Zone (adapted from NCCN Guidelines 2010) Staging tests: CT of Chest/abdominal/pelvic with contrast of diagnostic quality Bone marrow biopsy aspirate Evaluation for extranodal primary sites: Neck nodes: ocular[lymphomation.org]
Lymphocytic Infiltrate
  • A biopsy specimen and immunohistochemical analysis of the left arm lesions showed a lymphocytic infiltrate which stained positively for CD20 and Bcl-2 and negatively for CD10.[ncbi.nlm.nih.gov]
  • Clinical Features: Recurrent red to brown papules or plaques Most common on the upper extremities and trunk Possible association with Borrelia burgdorferi infection Excellent prognosis Histologic Features: Nodular to diffuse lymphocytic infiltrate involving[dermpedia.org]
  • The biopsy showed a top-heavy atypical lymphocytic infiltration with a Grenz zone. Polymerase chain reaction assays for Borrelia DNA were positive both from peripheral blood and biopsy.[ijdvl.com]
  • Contact Dermatitis 2:139–143 CrossRef PubMed Google Scholar Weiterführende Literatur zu lymphocytic infiltration of the skin Higgins CR, Wakeel RA, Cerio R (1994) Childhood Jessner's lymphocytic infiltrate of the skin.[doi.org]

Treatment

  • Surgical excision, local radiotherapy, or a combination were the initial treatment in 118 patients (86%).[ncbi.nlm.nih.gov]
  • Recurring tumors have worse prognosis compared to tumors that do not recur Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment Progression of the condition makes the outcome[dovemed.com]
  • Surgical excision, local radiotherapy, or combinations of both are the first-line treatment in solitary or localized disease.[bloodjournal.org]

Prognosis

  • Although prognosis is generally good, the disease has potential for skin as well as extracutaneous recurrences. Large cell transformation and head and neck presentation may be associated with a worse prognosis.[ncbi.nlm.nih.gov]

Etiology

  • Borrelia burgdorferi is a suspected etiologic agent identified in a subset of cases. Although all of these neoplasms presumably are monoclonal, monoclonal IgH rearrangement can only be detected in approximately 75% of cases.[ncbi.nlm.nih.gov]
  • Karger AG, Basel References Hovmark A, Asbrink E, Olsson I: The spirochetal etiology of lymphadenosis benigna cutis solitara. Acta Derm Venereol 1986;66:479–484.[karger.com]
  • Malignant B cells expanding marginal zones surrounding reactive follicles and colonizing them May be difficult to diagnose because of nonspecificity of many of the morphologic features, absence of 1 or more classic features, and nature of the small biopsies ETIOLOGY[basicmedicalkey.com]
  • Although its molecular pathogenesis is currently unknown, an etiological link with Borrelia burgdorferi infection has been identified in European, but not in American or Asian cases.[nature.com]

Epidemiology

  • Clinical Epidemiology. 2010. ( 2): 179-186 Dal Maso L, Franceschi S. Hepatitis C virus and risk of lymphoma and other lymphoid neoplasms: a meta-analysis of epidemiologic studies.[sapd.es]
  • […] zone lymphoma Autoimmune Diseases Marginal zone lymphoma has been associated with patients who have autoimmune diseases Sjögren syndrome and parotid gland marginal zone lymphoma Hashimoto thyroiditis and thyroid marginal zone lymphoma CLINICAL ISSUES Epidemiology[basicmedicalkey.com]
  • Epidemiology : occurs mostly in adults, but may occasionally affect children and adolescents.[ufrgs.br]
  • Intralesional rituximab in the treatment of indolent primary cutaneous B-cell lymphomas: an epidemiological observational multicenter study. The Spanish Working Group on Cutaneous Lymphoma. Br J Dermatol 2012; 167: 174–179. 9.[medicaljournals.se]
  • The epidemiology of non-Hodgkin's lymphoma: comparison of nodal and extra-nodal sites. Int J Cancer. 1997 Sep 17. 72(6):923-30. [Medline]. Gallo E, Pérez-Gala S, Navarro R, Fraga J, Adrados M, Arranz R, et al.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Similarly, the suspected pathophysiology for the induction of lymphoma by Borrelia is chronic antigen-dependent immunostimulation triggering sustained lymphoid proliferation with oligoclonal and ultimately monoclonal B-cell selection. [4] It has been[ijdvl.com]
  • Pathophysiology The development of cutaneous lymphoma is only partially understood. Most probably it is a multifactorial and multistep process, with various etiologic factors intervening over a long period.[emedicine.medscape.com]

Prevention

  • How can Primary Cutaneous Marginal Zone B-Cell Lymphoma be Prevented? Presently, the cause of Primary Cutaneous Marginal Zone B-Cell Lymphoma is unknown and there are no known methods to prevent its formation.[dovemed.com]
  • IRB 14321: A Single-Arm, Open-Label, Multicenter, Study of Folotyn (Pralatrexate Injection) in Combination with Oral Leucovorin to Prevent or Reduce Mucositis in Patients with Relapsed or Refractory (R/R) Peripheral T-Cell Lymphoma (PTCL).[cityofhope.org]

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