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Haematologica Dec 2023While all peripheral T-cell lymphomas are uncommon, certain subtypes are truly rare, with less than a few hundred cases per year in the USA. There are often no dedicated... (Review)
Review
While all peripheral T-cell lymphomas are uncommon, certain subtypes are truly rare, with less than a few hundred cases per year in the USA. There are often no dedicated clinical trials in these rare subtypes, and data are generally limited to case reports and retrospective case series. Therefore, clinical management is often based on this limited literature and extrapolation of data from the more common, nodal T-cell lymphomas in conjunction with personal experience. Nevertheless, thanks to tremendous pre-clinical efforts to understand these rare diseases, an increasing appreciation of the biological changes that underlie these entities is forming. In this review, we attempt to summarize the relevant literature regarding the initial management of certain rare subtypes, specifically subcutaneous panniculitis-like T-cell lymphoma, hepatosplenic T-cell lymphoma, intestinal T-cell lymphomas, and extranodal NK/T-cell lymphoma. While unequivocally established approaches in these diseases do not exist, we make cautious efforts to provide our approaches to clinical management when possible.
Topics: Humans; T-Lymphocytes; Retrospective Studies; Killer Cells, Natural; Lymphoma, T-Cell, Peripheral; Lymphoma, Extranodal NK-T-Cell
PubMed: 38037801
DOI: 10.3324/haematol.2023.282717 -
JAAD Case Reports Dec 2023
PubMed: 38034365
DOI: 10.1016/j.jdcr.2023.09.031 -
Oxford Medical Case Reports Nov 2023COVID-19 has many complications that affect many systems, including rheumatology and inflammatory skin conditions such as cutaneous lupus erythematosus. Herein, we...
COVID-19 has many complications that affect many systems, including rheumatology and inflammatory skin conditions such as cutaneous lupus erythematosus. Herein, we describe the case of a patient with lupus panniculitis who presented with systemic lupus erythematosus in the setting of recent COVID-19 infection. A 66-year-old female patient presented to the hospital with expanded skin lesions all over her limbs, fever, joint pain, and fatigue. Lab tests and imaging revealed a second recent infection with COVID-19, positive titers of systemic lupus erythematosus antibodies, and biopsy confirmed lupus erythematosus panniculitis. She was treated with oral prednisone and hydroxychloroquine for SLE and symptomatic management for recent COVID-19 infection without ICU admission. Lupus erythematosus panniculitis (LEP) is a rare manifestation of lupus erythematosus. Although some cases of SLE following COVID-19 infection have been reported, lupus panniculitis as the initial presentation of systemic lupus erythematosus in these patients is extremely rare.
PubMed: 38033407
DOI: 10.1093/omcr/omad129 -
Clinical Case Reports Nov 2023Erythema nodosum (EN) is a type of panniculitis occurring due to various conditions. It can be associated with certain malignancies or manifest as a side effect of...
Erythema nodosum (EN) is a type of panniculitis occurring due to various conditions. It can be associated with certain malignancies or manifest as a side effect of drugs. This article presents a unique case of EN in a patient with chronic myeloid leukemia (CML-blast phase) following dasatinib and chemotherapy. Timely recognition and appropriate management are crucial to alleviate symptoms and consider potential drug-induced etiology.
PubMed: 38028081
DOI: 10.1002/ccr3.8223 -
International Medical Case Reports... 2023An uncommon variation of lupus erythematosus is lupus panniculitis. İt can exist on its own or in conjunction with discoid or systemic lupus erythematosus. Persistent,...
An uncommon variation of lupus erythematosus is lupus panniculitis. İt can exist on its own or in conjunction with discoid or systemic lupus erythematosus. Persistent, sensitive, and hard nodules that are localized on the face, arms, shoulders, breast, and buttocks are its defining features. Scarring, lipoatrophy, and ulceration are occasionally associated with the healing of lesions. We are reporting the first case of lupus erythematosus panniculitis from Somalia. A 60-year-old male patient visited our polyclinic with an upper back nodule and a left upper ulcerated lesion that had been present for four months.
PubMed: 37954090
DOI: 10.2147/IMCRJ.S413740 -
The American Journal of Case Reports Nov 2023BACKGROUND An 82-year-old woman presented with acute pyrexial illness and mesenteric panniculitis and developed biochemical aseptic meningitis (cerebrospinal fluid...
BACKGROUND An 82-year-old woman presented with acute pyrexial illness and mesenteric panniculitis and developed biochemical aseptic meningitis (cerebrospinal fluid pleocytosis with no identifiable pathogen). Investigation determined her illness was likely a delayed hypersensitivity reaction caused by sulfasalazine. Sulfasalazine-induced aseptic meningitis is a rare condition often diagnosed late in a patient's admission owing to initial non-specific illness symptomatology requiring the exclusion of more common "red flag" etiologies, such as infection and malignancy. CASE REPORT An 82-year-old woman with a history of recurrent urinary tract infections and seronegative arthritis presented with a 3-day history of fatigue, headache, dyspnea, and lassitude. On admission, she was treated as presumed sepsis of uncertain source owing to pyrexia and tachycardia. Brain computer tomography (CT) revealed no acute intracranial abnormality. Furthermore, CT of the chest, abdomen, and pelvis did not reveal any source of sepsis or features of malignancy. After excluding infective etiologies with serological and cerebrospinal fluid testing, sulfasalazine-induced aseptic meningitis (SIAM) was diagnosed. The patient was then commenced on intravenous steroids, resulting in immediate defervescence and symptom resolution. CONCLUSIONS SIAM remains a diagnostic challenge since patients present with non-specific signs and symptoms, such as pyrexia, headaches, and lassitude. These patients require a thorough investigative battery starting with anamnesis, physical examination, biochemical testing, and radiologic imaging. This case illustrates the need for a high suspicion index of drug-induced hypersensitivity reaction in a rheumatological patient with pyrexial illness where infective etiologies have been confidently excluded. Prompt initiation of intravenous steroids in SIAM provides a dramatic recovery and resolution of symptoms.
Topics: Female; Humans; Aged, 80 and over; Meningitis, Aseptic; Sulfasalazine; Panniculitis, Peritoneal; Arthritis; Fever; Sepsis; Neoplasms; Fatigue; Hypersensitivity, Delayed; Steroids
PubMed: 37924204
DOI: 10.12659/AJCR.941623 -
Medicine Oct 2023Subcutaneous panniculitis like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma that belongs to peripheral T cell lymphomas, of which the overall prognosis...
RATIONALE
Subcutaneous panniculitis like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma that belongs to peripheral T cell lymphomas, of which the overall prognosis is poor. Chidamide, a deacetylase inhibitor, has been approved for the treatment of peripheral T cell lymphomas. However, due to the rare occurrence of SPTCL, it is currently unknown whether Chidamide is effective for all SPTCL patients and whether there are molecular markers that can predict its therapeutic effect on SPTCL.
PATIENT CONCERNS AND DIAGNOSES
The patient was a sixteen-year-old male and underwent subcutaneous nodule biopsy which showed SPTCL. Next-generation sequencing revealed AT-rich interaction domain 1A (ARID1A) mutation, and positron emission tomography/computed tomography showed scattered subcutaneous fluorodeoxyglucose metabolic lesions throughout the body.
INTERVENTIONS AND OUTCOMES
During the first 3 CHOP (cyclophosphamide, doxorubicin, vindesine, and prednisone) treatment, the patient relapsed again after remission, and the successive addition of methotrexate and cyclosporine did not make the patient relapsing again. Then, after adding Chidamide to the last 3 CHOP treatment, the patient was relieved again. The patient underwent autologous hematopoietic stem cell transplantation (auto-HSCT) after completing a total of 8 cycles of chemotherapy, and continued maintenance therapy with Chidamide after auto-HSCT. Currently, the patient has been in continuous remission for 35 months.
LESSONS SUBSECTIONS
This case is the first report of a refractory/recurrent SPTCL with ARID1A mutation treated with Chidamide. The treatment of Chidamide on the basis of CHOP plus auto-HSCT therapy achieved good results, suggesting that ARID1A may act as a molecular marker to predict the therapeutic effect of Chidamide on SPTCL patients, which helps to improve the precision of SPTCL treatment and the overall prognosis of SPTCL patients.
Topics: Male; Humans; Adolescent; Lymphoma, T-Cell, Peripheral; Neoplasm Recurrence, Local; Lymphoma, T-Cell; Hematopoietic Stem Cell Transplantation; Panniculitis; Mycosis Fungoides; Skin Neoplasms; Antineoplastic Combined Chemotherapy Protocols; DNA-Binding Proteins; Transcription Factors
PubMed: 37800816
DOI: 10.1097/MD.0000000000035413 -
Skin Health and Disease Oct 2023Sclerema neonatorum (SN) is a rare condition of neonatal panniculitis with a poor prognosis and a high fatality rate. It clinically presents as hardening of the skin and...
Sclerema neonatorum (SN) is a rare condition of neonatal panniculitis with a poor prognosis and a high fatality rate. It clinically presents as hardening of the skin and subcutaneous adipose tissue extending throughout the body, sparing the fat-free soles, palms, and genitalia. SN typically affects gravely ill, preterm neonates in the first week of life and diagnosis is often clinical. We report a case of an eight-day-old premature infant diagnosed with early-onset neonatal sepsis who presented with clinical and histopathological features of SN. Despite early treatment of the sepsis with intravenous antibiotics and the SN with a topical corticosteroid cream and moisturiser, the infant died on the twelfth day of life.
PubMed: 37799354
DOI: 10.1002/ski2.255 -
ACR Open Rheumatology Jan 2024
PubMed: 37779254
DOI: 10.1002/acr2.11615 -
JAAD Case Reports Oct 2023
PubMed: 37771353
DOI: 10.1016/j.jdcr.2023.08.031