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Hydroa Vacciniforme-Like Lymphoma


Presentation

  • Our study presents a rare clinical variant of HVL with predominant periorbital edema.[ncbi.nlm.nih.gov]
  • Fang Published: 09 August 2016 A 35-year-old female presented with history of relapsing facial rash and fever since 3 years. The facial lesions progressed through small papules or vesicles before healing with atrophic scars.[academic.oup.com]
Intermittent Fever
  • According to the limited reports in the literature, the clinical course may be indolent in patients with the NK-cell phenotype; whereas cases with T-cell phenotype are characterized by intermittent fever and hepatosplenomegaly.[diagnosticpathology.biomedcentral.com]
Rigor
  • .: [4] (i) a self-limited, vesiculopapular eruption on the exposed areas; (ii) no systemic involvement is present and the disorder improves with rigorous protection from the sun; (iii) histological features of reticulated epidermal necrosis or blister[e-ijd.org]
Turkish
  • A 45-year-old Turkish woman presented with persistent swelling of the face, erosive facial papules and small oral ulcers that had developed 5 years previously ( Fig. 1 ).[medicaljournals.se]
Abdominal Lymphadenopathy
  • Computed tomography scan showed thickened intestinal wall, generalized abdominal lymphadenopathy and hepatosplenomegaly. The intestine histology was also consistent with hydroa vacciniforme-like lymphoma. She was still follow-up. Figure 1.[academic.oup.com]
Inguinal Lymphadenopathy
  • Cervical and inguinal lymphadenopathy were detected. The histopathology and immunohistochemistry of specimen from the skin showed epidermal ulceration accompanied by diffuse infiltrate with atypical lymphoid cells.[academic.oup.com]
Mediastinal Lymphadenopathy
  • Computed tomography (CT) scans revealed splenomegaly, cervical and mediastinal lymphadenopathy, hilar and para-aortal consolidations and oedematous ocular muscles. Fig. 1. Clinical features. (a) Oedematous swelling of the eyelids and the lips.[medicaljournals.se]
Sputum
Aspiration
  • This can also help determine, if the cancer has spread to other organ systems Brain MRIs are used if neurological symptoms are present, which can help determine if the cancer has spread to the brain, or to tissues that cover the brain Bone marrow aspiration[dovemed.com]
Abdominal Pain
  • […] liver (hepatomegaly) There may be 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[dovemed.com]
Constipation
  • […] temperatures and excessive night sweats (may be recurrent) Anemia (low red blood cell count) Low lymphocyte count, established by a blood test Frequent infections Trouble breathing Low blood pressure Back pain Swelling of the legs Abdominal pain and swelling; constipation[dovemed.com]
Nausea
  • Under such circumstances, removing all the affected lymph nodes would be extremely difficult Supportive treatment: Steroids, blood transfusions, anti-nausea medications, and antibiotics, may be used as supportive therapy.[dovemed.com]
Hematochezia
  • Although the rashes were relieved, she still complained of fever and presented with hematochezia. Computed tomography scan showed thickened intestinal wall, generalized abdominal lymphadenopathy and hepatosplenomegaly.[academic.oup.com]
Nikolsky's Sign
Facial Papule
  • A 45-year-old Turkish woman presented with persistent swelling of the face, erosive facial papules and small oral ulcers that had developed 5 years previously ( Fig. 1 ).[medicaljournals.se]
Periorbital Edema
  • Our study presents a rare clinical variant of HVL with predominant periorbital edema.[ncbi.nlm.nih.gov]
Diplopia
  • The clinical course was complicated by the development of diplopia and excessive B-symptoms (weight loss, night sweats and a reduced condition).[medicaljournals.se]
Facial Numbness
  • numbness If the abdomen is affected, it can cause intestinal obstruction that results in urine outflow obstruction and kidney damage There may be complications related to chemotherapy used in treating the condition, which may include: Side effects such[dovemed.com]

Workup

Enlargement of the Liver
  • Ultrasonic examination revealed slight enlargement of the liver and spleen. Chest X-ray was unremarkable.[e-ijd.org]
Pseudomonas
  • Sputum culture found Pseudomonas aeruginosa. Cervical lymph node biopsy was performed and systemic EBV-positive T-cell lymphoproliferative disease was diagnosed. Clinically, respiratory tract infection was also considered.[diagnosticpathology.biomedcentral.com]

Treatment

  • treatment and/or systemic treatment, when the whole body is affected In some children, the condition progresses rapidly, while in others the progress is slower.[dovemed.com]
  • A complete reference to the latest understanding of lymphocytic infiltrates The Cutaneous Lymphoid Proliferations provides a complete, authoritative treatment of the clinical features, phenotypes, pathogenesis, histopathology, diagnosis, and treatment[books.google.com]
  • The patients underwent glucocorticoid treatment and obtained remarkable clinical improvement with regression of skin lesions. No sign of recurrence and extracutaneous manifestation was found during the period of follow-up.[ncbi.nlm.nih.gov]
  • Treatment for patients with EBV-associated HV is not satisfactory.[e-ijd.org]

Prognosis

  • This report presents 2 cases of primary cutaneous HV-like lymphoma with unusual indolent clinical course and favorable prognosis during the follow-up periods of 2 and 3 years, respectively.[ncbi.nlm.nih.gov]
  • The prognosis of the condition is poor; only 1 in 3 survive after 2 years of diagnosis The prognosis depends upon a set of several factors, which include: Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis[dovemed.com]
  • The prognosis is poor.[tititudorancea.com]

Etiology

  • […] and Mexico Epidemiology Mainly children and adolescents from Asia, or Native Americans from Central and South America, and Mexico Rare in adults Sites Sun exposed skin, particularly the face (cheeks, nose, lower lip) but also ears and dorsum of hands Etiology[pathologyoutlines.com]
  • The etiology of the condition is still unknown; there is theory of an abnormal sensitivity to the UVA (Ultraviolet rays type A) radiation in a range of 320 to 390 nm of wavelength.[oxyhives.org]
  • Pathophysiology The etiology of hydroa vacciniforme (HV) is uncertain. HV may be a distinct entity distinguished by scarring or may occur within the spectrum of polymorphous light eruption.[emedicine.medscape.com]

Epidemiology

  • Occurs in children and associated with sun sensitivity ( WHO 2008 ) Increased frequency in Asians and Native Americans from Central and South America, and Mexico Epidemiology Mainly children and adolescents from Asia, or Native Americans from Central[pathologyoutlines.com]
  • Papulovesicular eruption resulting in ulceration and scarring Wasting Diagnostic Exams Flow cytometry Immunohistochemistry Immunophenotyping Progression and Transformation Recurrent skin lesion s for 10-15 years before progression to system ic involvement Epidemiology[seer.cancer.gov]
  • […] type Mycosis fungoides Precursor T-cell lymphoblastic lymphoma Peripheral T-cell lymphoma, not otherwise characterized Primary cutaneous anaplastic large cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma Lupus [10] Herpes simplex [11] [1] Epidemiology[wikidoc.org]
  • SURVIVAL IN PEDIATRIC SARCOMAS E León, L Vasquez, I Maza, M Oscanoa, J Gerónimo PEDIATRIC BLOOD & CANCER 63, S125-S125 , 2016 2016 Advancing Parental Age and Risk of Solid Tumors in Childhood: Evidence from a Case-Control Study and A Meta-Analysis of Epidemiological[scholar.google.com]
Sex distribution
Age distribution

Pathophysiology

  • Historical Perspective HVLL reports date back to 1986; [5] however, it is not until 2008 that the WHO recognized HVLL as a separate entity from Hydroa vacciniforme and classified it under "EBV positive T-cell lymphoproliferative disorders of childhood". [2] Pathophysiology[wikidoc.org]
  • These entities often pose diagnostic challenges, both clinically and pathologically, and it is important to understand their unique pathophysiology for correct diagnoses and optimal management.[jpatholtm.org]
  • Pathophysiology The etiology of hydroa vacciniforme (HV) is uncertain. HV may be a distinct entity distinguished by scarring or may occur within the spectrum of polymorphous light eruption.[emedicine.medscape.com]
  • Etiology Pathophysiology The pathogenesis of HV is not fully understood. Sunlight is required to provoke eruption, and in the temperate climate the onset is usually in spring or summer.[clinicaladvisor.com]
  • Pathophysiology and epidemiology of hemophagocytic lymphohistiocytosis. Hematol Am Soc Hematol Educ Program . (2015) 2015:177–82. doi: 10.1182/asheducation-2015.1.177 PubMed Abstract CrossRef Full Text Google Scholar 9.[frontiersin.org]

Prevention

  • How can Hydroa Vacciniforme-like Cutaneous T-Cell Lymphoma be Prevented? Currently, the cause of Hydroa Vacciniforme-like Cutaneous T-Cell Lymphoma is unknown. It may not be hence possible to prevent the tumor from forming.[dovemed.com]
  • Photoprotection may not prevent the skin lesions or systemic symptoms. [4] , [9] Immunosuppressive therapy, such as systemic corticosteroids or cyclosporine A has been used, but the disease usually flares after decreasing the dose, as occurred in this[e-ijd.org]
  • Narrow-band UVB (TL-01) phototherapy: an effective preventative treatment for the photodermatoses. Br J Dermatol. 1995 Jun. 132(6):956-63. [Medline]. Hashizume H, Tokura Y, Oku T, Iwamoto Y, Takigawa M.[emedicine.medscape.com]
  • Prevention of the deleterious effects of ultraviolet is most important—clothing, broad-spectrum sunscreens. The condition in its classic form remits usually during adolescence.[clinicaladvisor.com]

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