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ALK-Negative Anaplastic Large-Cell Lymphoma

ALK- ALCL


Presentation

Soft Tissue Mass
  • They should implement a broad panel of immunomarkers in soft-tissue masses of anaplastic pleomorphic cells with an alveolar morphology.[ncbi.nlm.nih.gov]
  • 29359238 ) Doghri R....Mrad K. 2018 8 Acquired ichthyosis as a paraneoplastic feature of ALK-negative anaplastic large cell lymphoma. ( 30407628 ) Valchev G...Shivarov V 2018 9 ALK-Negative Anaplastic Large Cell Lymphoma Presenting as an Extranodal Soft-Tissue[malacards.org]
  • Soft tissue masses may sometimes be mistaken for soft tissue sarcomas. Involvement of other extranodal sites is less commonly seen. ALCL has an aggressive natural history, but an excellent response to combination chemotherapy containing doxorubicin.[nature.com]
Constitutional Symptom
  • A 55-year-old man with a history of well controlled HIV infection was admitted with acute renal impairment, peripheral oedema, constitutional symptoms, deranged liver function and hypercalcaemia.[ncbi.nlm.nih.gov]
  • symptoms, deranged liver function and hypercalcaemia.[casereports.bmj.com]
Cape Verde
  • Cape Verde : Boa Vista Cape Verde, Sal, etc. Caribbean Netherlands :, etc. Cayman Islands : George Town, Grand Cayman, West Bay, etc. Central African Republic : Bangui, etc. Chad : N'Djamena, etc.[maria-online.com]
Fever of Unknown Origin
  • Mosunjac, “Unusual Presentation of Anaplastic Large Cell Lymphoma with Clinical Course Mimicking Fever of Unknown Origin and Sepsis: Autopsy Study of Five Cases,” Croatian Medical Journal, Vol. 49, No. 5, 2008, pp. 660-668. doi:10.3325/cmj.2008.5.660[scirp.org]
Heat Intolerance
  • […] include: Non-painful swelling of lymph nodes in the neck, armpits, or groin; the lymphoma may occur in the lymph nodes and at extra lymph nodes (extranodal locations) There may be associated autoimmune disorders, which can cause joint and muscle pain, heat[dovemed.com]
Tinnitus
  • […] intolerance, recurrent rashes, abdominal pain, and a general feeling of illness Bone marrow involvement can be seen in 1 in 4 individuals (25% of the cases) 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]
Neck Mass
  • We report a case of ALK-negative ALCL that presented as an extranodal soft-tissue neck mass in an adult man.[ncbi.nlm.nih.gov]
  • Right neck mass needle core biopsy showing intermediate to large cells with anaplastic morphology, including hallmark cells (black arrows). H&E staining at 500 magnification.[captodayonline.com]
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]
Stroke
  • […] 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 and cause clogging of vessels resulting in stroke[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]
Dizziness
  • […] rise to severe anemia The leukemic cells can overgrow and cause clogging of vessels resulting in stroke, or blurring of vision There may be complications related to chemotherapy used in treating the condition, which may include: Side effects such as dizziness[dovemed.com]

Workup

  • Distinguishing primary cutaneous anaplastic lymphoma (PC-ALCL) from its systemic counterpart requires a complete clinical and laboratory workup.[cancerjournal.net]
  • Workup A PET/CT scan showed increased FDG uptake in the remaining subpectoral node as well as the lung nodule. Fine-needle aspiration of the lung nodule showed an anaplastic large cell lymphoma, with large CD30-positive T cells.[ascopost.com]
  • Gallium Scanning Gallium scans, although not as useful in the initial staging workup, can be helpful for evaluating the patient’s response to treatment, because continued positive uptake in a residual mass after completion of treatment is an indicator[emedicine.medscape.com]

Treatment

  • Bortezomib, a reversible proteasome inhibitor, is used for the treatment of hematologic malignancies.[ncbi.nlm.nih.gov]
  • Treatment with bortezomib resulted in proteasome inhibition of more than 80% in all primary ALCL cells and ALCL cell lines tested.[haematologica.org]
  • 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]

Prognosis

  • ALCL and PTCL-NOS or AILT is of limited clinical relevance as they show comparable poor prognosis.[ncbi.nlm.nih.gov]
  • The prognosis is typically poor Generally, at the time of diagnosis, ALK-Negative ALCLs are at an advanced-stage or higher-stage; this makes the prognosis worse.[dovemed.com]

Etiology

  • […] old Male predominance (M:F ratio 1.5:1) Most epidemiologic studies do not separate ALK from ALK- neoplasms Sites Frequent involvement of both lymph nodes and extranodal sites Usually nodal; extranodal involvement in skin, bone, soft tissue, GI tract Etiology[pathologyoutlines.com]
  • Etiology The etiology of ALCL, ALK negative is unknown.[atlasgeneticsoncology.org]
  • Studies reported that the etiology of this NHL may comprise hereditary, environmental, occupational and dietary factors.[thejh.org]
  • (Etiology) Lymphocytes are a type of white blood cells that are responsible for providing immunity in the human body. B-cells and T-cells are the two different types of lymphocytes.[dovemed.com]
  • Due to limited number of cases, the etiology for aggressive nature of this presentation is not well studied. More than half of the reported cases have complex karyotype. Our patient had typical translocation t(2; 5) with no additional abnormalities.[omicsonline.org]

Epidemiology

  • Most frequently 40 - 65 years old Male predominance (M:F ratio 1.5:1) Most epidemiologic studies do not separate ALK from ALK- neoplasms Sites Frequent involvement of both lymph nodes and extranodal sites Usually nodal; extranodal involvement in skin[pathologyoutlines.com]
  • Pain in the chest, abdomen, or bone s (for no known reason) Pruritis Weight loss (for no known reason) Diagnostic Exams Flow cytometry Immunohistochemistry Immunophenotyping Progression and Transformation Frequent relapses of disease after treatment Epidemiology[seer.cancer.gov]
  • Epidemiology ALCL, ALK-negative makes up between 2.6% and 9.4% of T-/NK-cell non-Hodgkin lymphomas, with the lowest proportion occurring in Asia and the highest in Europe (Vose, Armitage et al. 2008).[atlasgeneticsoncology.org]
  • Anderson JR, Armitage JO, Weisenburger DD (1998) Epidemiology of the non-Hodgkin’s lymphomas: distributions of the major subtypes differ by geographic locations Non-Hodgkin’s Lymphoma Classification Project.[link.springer.com]
  • This tumor can be recurrent and grow as a mass around the implant capsule or can extend to regional lymph nodes if not properly treated. [9] Epidemiology [ edit ] The FDA recorded over 450 cases of ALCL due to breast implants between 2010 and 2018. [17[en.wikipedia.org]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology ALK positive and ALK negative ALCL have different gene expression profiles and seem to be biologically distinct entities. The pathophysiology of both subtypes of ALCL is incompletely understood.[cancertherapyadvisor.com]
  • […] thought to play a central role in the regulation of many diverse aspects of cell biology; however, it remains to be fully elucidated how microRNAs can orchestrate cellular redox homeostasis, which plays a central role in a multitude of physiological and pathophysiological[cancer-genetics.org]

Prevention

  • How can ALK-Negative Anaplastic Large Cell Lymphoma be Prevented? Presently, the cause of ALK-Negative Anaplastic Large Cell Lymphoma is unknown. Therefore, there are no known methods to prevent its occurrence.[dovemed.com]
  • […] cell lymphomas and has skin-homing properties; therefore, it is postulated that it limits the extracutaneous spread of the disease. 4 The association between the two genes may be related to these more favorable outcomes secondary to the homing signals preventing[captodayonline.com]
  • This difference is probably caused by expression in ALK-positive ALCL of heat shock protein 72 (HSP72) that prevents Bax activation, inhibits the release of cytochrome c from the mitochondria and limits downstream caspase-3 activity. 9 HSP72 is involved[haematologica.org]
  • Chen Tian, Yizhuo Zhang Department of Hematology, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin, People’s Republic of China Introduction[dovepress.com]
  • Thus, a strategy that prolongs relapse-free survival or prevents relapse is desirable.[jhoponline.com]

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