Presentation
Features contributing to the diagnostic conundrum in this case included an atypical clinical and histological presentation, lack of pertinent clinical history and multiple presentations at different institutions. [ncbi.nlm.nih.gov]
Entire Body System
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Falling
[…] in reporting this case is to emphasize that in the context of primary cutaneous B-cell lymphoma, the 9p21 deletion while characteristic of the leg-type lymphoma is not a unique signature of the leg-type lymphoma and is not exclusionary to lymphomas falling [ncbi.nlm.nih.gov]
Skin lymphomas that don't fall into any of these three categories are called primary cutaneous peripheral T-cell lymphoma, unspecified. [seattlecca.org]
The treatments mentioned in this section fall into 2 main groups: Skin-directed treatments Whole-body (systemic) treatments T-cell lymphomas Mycosis fungoides (MF) Many forms of treatment can be used for MF. [cancer.org]
Skin
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Papule
The last patient had miliary papules on the entire face, back, upper aspect of arms, and scattered on the front of the chest. [ncbi.nlm.nih.gov]
Clinical Features: Presents as solitary or grouped papules, plaques or tumors Most common on the scalp, forehead and back Good prognosis Histologic Features: Nodular or diffuse infiltrate occupying the entire dermis and sometimes extending into the subcuataneous [dermpedia.org]
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Subcutaneous Nodule
A 62-year-old man presented with a solitary indolent subcutaneous nodule for 3 years duration, without other abnormalities. [ncbi.nlm.nih.gov]
Whole-body Positron Emission Tomography--Computed Tomography scan revealed a 1.9 cm subcutaneous nodule in the anteromedial soft tissues of the left thigh with marked increased radiotracer uptake. [ijdvl.com]
Clinical Features : Multiple subcutaneous nodules involving trunk and extremities, rarely, the face. Constitutional symptoms : fever, fatigue, and weight loss may be present. Pancytopenia : usually, due to cytokine mediated BM suppression [slideshare.net]
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Skin Papule
Seven patients with cutaneous B-cell pseudolymphoma (2 males, 5 females; mean age, 36.3 years; range, 9 to 60 years) had solitary, erythematous skin papules or small nodules located on the face (n 1), ear (n 3), trunk (n 2), or upper extremities [bloodjournal.org]
You may have small, raised, solid areas of skin ( papules) or flatter, thickened areas of skin (plaques). Some people have larger lumps called nodules or tumours, which are often deep-red or purplish in colour. They can ulcerate and become infected. [lymphomas.org.uk]
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Skin Plaque
You may have small, raised, solid areas of skin ( papules) or flatter, thickened areas of skin (plaques). Some people have larger lumps called nodules or tumours, which are often deep-red or purplish in colour. They can ulcerate and become infected. [lymphomas.org.uk]
Musculoskeletal
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Long Arm
We report a reciprocal translocation between the long arms of chromosomes 12 and 21, t(12;21)(q13;q22), in a patient with primary cutaneous follicle center lymphoma. [ncbi.nlm.nih.gov]
The other interesting finding in this case is the deletion present on the long arm of one chromosome 10. [path.upmc.edu]
arms of chromosomes 12 and 21, t(12;21)(q13;q22), has been found in a patient with primary cutaneous FCL, [67] which again may suggest a potential pathogenetic pathway of these tumors, but so far has no practical diagnostic relevance. [emedicine.medscape.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]
For further workup, a 6 mm punch biopsy was done from the lesion. Histologically, the skin biopsy showed an unremarkable epidermis. [ijpmonline.org]
Other Pathologies
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Lymphocytic Infiltrate
Punch biopsy taken from a left arm skin lesion showed a diffuse and nodular large cell lymphocytic infiltrate in the 15-20 μm range exhibiting round to oval nuclei and prominent eosinophilic nucleoli. [ncbi.nlm.nih.gov]
infiltrate •Chronic lymphocytic leukaemia, B cell type•Inflammatory pseudotumour •Plasmacytoma •Marginal zone B-cell lymphoma 89. [slideshare.net]
Treatment
Due to a refusal to treatment, the lesion progressively grew until now. [ncbi.nlm.nih.gov]
In patients who do require some treatment intervention, complete surgical excision of the lesion or lesions in question is common practice, as is radiotherapy. [lymphomainfo.net]
You can help by adding to it. ( May 2018 ) Treatment [ edit ] Surgical removal and/or radiotherapy is given for localized disease. [2] : 314 [6] Radiation using multiple radiation fields is given if the disease has wider extent with grouped lesions. [ [en.wikipedia.org]
Prognosis
The distinction between primary and secondary B-cell lymphomas is essential for defining prognosis/course of action. [ncbi.nlm.nih.gov]
The relapse rate is about 30 percent, but often relapse does not correlate with prognosis. Where prognosis gets a little tricky is on the legs. [lymphomainfo.net]
Etiology
[…] with the presence of bcl-6 and occasional network of follicular dendritic cells in the background. [4] An association with Borrelia burgdorferi, hepatitis C, or human herpesvirus 8 has been described in sporadic patients but does not seem to be a major etiological [bioline.org.br]
The etiology may involve microbial, physical, chemical, Borrelia burgdorferi infection, insect bite, tattoo, and drugs Clinically, several variants of C-PSL exist. .... [lymphomation.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]
Association with infections such as Borrelia burgdorferi, hepatitis C, or human herpesvirus 8 (HHV-8) has been described in sporadic patients, but does not seem to be an important etiologic factor for cutaneous follicle center lymphoma [15, 16]. [plasticsurgerykey.com]
Most probably it is a multifactorial and multistep process, with various etiologic factors intervening over a long period. The disease likely begins as a hyperreactive inflammatory process. [emedicine.medscape.com]
Epidemiology
B-cell lymphoma of follicle center cells, primary in skin Disease limited to skin for 6 months after diagnosis Distinct disease from systemic follicular lymphoma Better prognosis Fewer BCL2 rearrangements than systemic follicular lymphoma CLINICAL ISSUES Epidemiology [basicmedicalkey.com]
Immunohistochemistry Immunophenotyping Progression and Transformation Cutaneous relapses noted in 30% of patients Recurrences usually proximate to the initial site of the tumor Untreated skin lesion s increase in size, rarely metastases to other site s Epidemiology [seer.cancer.gov]
considered indolent instead of aggressive) Epidemiology [ edit ] Overall (data from SEER, PMID 15908651 ) Mean age 64 yrs. Male 54%. [en.wikibooks.org]
Epidemiology : occurs mostly in adults, but may occasionally affect children and adolescents. [ufrgs.br]
Peñate Y, Hernández-Machín B, Pérez-Méndez LI, Santiago F, Rosales B, et al. (2012) Intralesional rituximab in the treatment of indolent primary cutaneous B-cell lymphomas: an epidemiological observational multicentre study. [omicsonline.org]
Pathophysiology
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
Skin-directed therapies Topical chemotherapy – creams containing drugs which may be used to kill and prevent the growth and division of cancer cells Phototherapy UVB – broad and narrow band, and PUVA – the use of ultraviolet light to treat the skin Radiation [leukaemia.org.au]
How can Primary Cutaneous Follicular Center Lymphoma be Prevented? Presently, the cause of Primary Cutaneous Follicular Center Lymphoma is unknown and there are no known methods to prevent its formation. [dovemed.com]
Microdissection was performed as follows: 4-μm sections cut from formalin-fixed, paraffin-embedded tissues were mounted on glass slides with a thickness of 0.17 mm (very thin glass slides are needed to prevent laser energy from being dispersed before [bloodjournal.org]
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]