Subcutaneous panniculitis-like T-cell lymphoma (SPTL) of the α/β type is a rare subtype of non-Hodgkin's lymphoma of the skin. Although these tumors usually run an indolent course, disease-related morbidity is often severe. Clinical findings include subcutaneous tumors located on the extremities or trunk, often accompanied by systemic symptoms like fever or fatigue. Due to the low incidence of SPTL, no standardized therapy has been defined so far and there is currently no curative therapy available for this type of non-Hodgkin's lymphoma. By sharing our experience with bexarotene therapy, we present a safe and potentially improved treatment for patients with SPTL. In the case presented, bexarotene was able to induce remission even after recurrence of disease.
Cutaneous T-cell lymphoma; Lymphoma; Panniculitis; Therapy; Retinoids; Bexarotene
Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a very rare postthymic T-cell non-Hodgkin's lymphoma with poor prognosis. There is not a standard treatment for this disease. Here we describe the first case of SPTL with unusual periorbital involvement, pancytopenia, hepatic dysfunction and coagulopathy, which was successfully treated with a chemotherapy regimen of cyclophosphamide, hydroxydaunomycin (doxorubicin), Oncovin (vincristine) and prednisone (CHOP). Our case demonstrates that although the natural history of SPTL is aggressive, patients may respond effectively to combination chemotherapy. Early recognition of the classic subcutaneous lesions and its associated systemic signs, such as unusual periorbital involvement, liver dysfunction and hemophagocytic syndrome, is very important in managing this aggressive lymphoma. Immunohistochemical and genetic studies are helpful in confirming the diagnosis. Early initiation of aggressive chemotherapy is recommended for better clinical outcome.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare T-cell lymphoma characterized by involvement of the subcutaneous tissue of neoplastic T lymphocytes. SPTCL with hemophagocytic syndrome (HPS) is associated with an aggressive clinical course and treatment of SPTCL with HPS is not well established. Cyclophosphamide, doxorubicin, vincristine, prednisolone (CHOP) therapy is not successful in most patients suffering from SPTCL with HPS. The role of high dose chemotherapy followed by hematopoietic stem cell transplantation (HSCT) remains controversial. We report a case of relapsed SPTCL after CHOP chemotherapy and salvage chemotherapy followed by autologous HSCT, which had rapid improvement within weeks after cyclosporine and prednisolone. Immunosuppressive therapy may be an important and successful treatment option in SPTCL patients, even though they may have clinically aggressive disease.
Panniculitis; Lymphoma; T-lymphocytes; Cyclosporine
Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a distinctive skin lymphoma characterized by neoplastic T-cell infiltration of the subcutaneous tissue, mimicking panniculitis.
To describe the clinical and pathologic features of SPTL in Korean patients.
Fourteen SPTL patients evaluated over 15 years were retrospectively reviewed.
The mean patient age was 35 years (range: 7~73 years), with male predominance (2.5:1). Most patients presented with either nodules or plaques, occurring most commonly on the trunk, with two patients (14%) having hemophagocytic syndrome. Histopathologically, all patients showed infiltrates of small-to-medium pleomorphic cells mimicking panniculitis, with some also showing rimming, bean-bag cells, and fat necrosis. Most patients were positive for CD3 (14/14), CD8 (12/13), TIA-1 (9/9) and βf1 (5/5), but were negative for CD4 (11/12), CD20 (8/8), CD56 (14/14) and Epstein-Barr virus (8/8). Ten patients (71%) received chemotherapy and 2 (14%) died due to the disease, with an average survival time of 4 months. Survival analysis did not reveal any significant prognostic factors.
This is the first series of patients with SPTL in Korea. Due to its indolent clinical course and relatively high survival rate, SPTL should be differentiated from cutaneous γδ T-cell lymphoma.
Subcutaneous panniculitis-like T-cell lymphoma
A 25-year-old man was diagnosised subcutaneous panniculitis-like T-cell lymphoma (SPTCL) through biopsy of a nodule from the anterior chest. After the treatment with prednisone 90 mg 3 weeks and tapered off in 1 month, the disease released, but relapsed together with symptions of hemophagocytic syndrome eight months after the termination of prednisone. CHOEP recipe was given but with unsatisfactory result until cyclosporine was prescribed. Cyclosporine was removed 6 months later. There is no evidence of clinical relapse 1 year later. This case suggest that cyclosporine could be a selectable treatment even in relapsed SPTCL.
relapsed; subcutaneous panniculitis-like T-cell lymphoma; cyclosporin A.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of cutaneous lymphomas, accounting for less than 1% of cases of non-Hodgkin’s lymphoma. Fluorine-18 fluorodeoxyglucose (F-18 FDG) positron-emission tomography/computed tomography (PET/CT) findings of SPTCL before and after treatment were rarely reported.
We report a case of SPTCL in which F-18 FDG PET/CT showed increased FDG accumulations in numerous subcutaneous nodules without extracutaneous disease. Contrast-enhanced CT during F-18 FDG PET/CT showed multiple minimally enhancing nodules with an infiltrative pattern in the subcutaneous layer throughout the body. Follow-up F-18 FDG PET/CT after three cycles of CHOP chemotherapy showed a complete metabolic remission of the lesions.
F-18 FDG PET/CT is suggested to be useful in assessing the disease activity, extent and treatment response in SPTCL.
subcutaneous panniculitis-like T-cell lymphoma; F-18 FDG; PET/CT
Subcutaneous panniculitis-like T-cell lymphomas (SPTLs) α/β are rare in childhood. The present report refers to a case of a 7-year-old male child presenting an extensive skin lesion that began when he was 5 years of age. Two biopsies were evaluated using the CD3, CD4, CD8, CD56, βF1, and TIA markers. A dense infiltrate of CD3+, CD4−, CD8+, CD56−, βF1+, and TIA+ pleomorphic lymphocytes was found in the subcutis. The previous biopsy showed cytophagic histiocytic panniculitis with a small focus on CD8+ and βF1+ malignant cells. The lesion regressed spontaneously. This case shows that prognosis may be excellent in SPTL (α/β). On the other hand, it also serves as an alert that a biopsy performed in an area of cytophagic panniculitis may lead to misdiagnosis.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon extra nodal non-Hodgkin lymphoma accounting for less than 1% of all NHLs known to have an aggressive course, with no well-defined treatment protocols. A 42-year-old lady, operated five months earlier for a squamous cell carcinoma of the cervix presented with pain and induration of the lower part of the anterior abdominal wall; 3 months after completing chemotherapy and radiotherapy. FNAC done, yielded scanty material and was inconclusive. The biopsy showed features of a subcutaneous panniculitis-like T-cell lymphoma. While on chemotherapy she developed a vault recurrence and extensive intra-abdominal spread of the squamous cell carcinoma and succumbed. SPTCL is a rare entity and has been reported in renal and cardiac allograft recipients and in one case of ovarian carcinoma. Its occurrence in the setting of carcinoma cervix is unusual, hence is being reported.
Carcinoma cervix; panniculitis; T-cell lymphoma
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a very rare form of skin lymphoma that is localized primarily to the subcutaneous adipose tissue without palpable involvement of the lymph nodes. Diagnosis of SPTCL is a challenge, especially during its early phases when symptoms mimic other, more common conditions, such as benign panniculitis, eczema, dermatitis, psoriasis and cellulitis. Clinical and systemic features are nonspecific and can include fever, chills, and weight loss. Further complicating diagnosis is the high number of false negatives provided by biopsy. Here we present a case of SPTCL that illustrates the full course of the disease, from presentation and multiple misdiagnoses to correct disease recognition and successful treatment. A review of the challenges of diagnosis is provided with recommendations for more accurate and timely recognition of SPTCL.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of cutaneous lymphoma. There have been a few case reports describing the radiologic imaging findings of SPTCL. We report a case of SPTCL, rarely presented with a breast mass. Here, we review her clinical history and radiologic (mammography and ultrasound) findings.
Subcutaneous panniculitis-like T-cell lymphoma; Breast; Mammography; Ultrasound
Panniculitis-like T-cell lymphoma is a very uncommon subtype of cutaneous T-cell lymphomas. In this case report, we describe the morphological (CT and MRI) and functional (18F-FDG-PET and bone scan) imaging findings in a 35-year-old patient who suffered from slowly progressing multiple subcutaneous lesions caused by this rare disease.
This case report describes a 38 year-old lady with the clinical, histopathological, and immunohistochemical (IHC) changes of subcutaneous panniculitis-like T-cell lymphoma (SPTCL). The IHC findings revealed CD8 + and CD56 – cells, which are indicative of tumors which have an indolent course. Our patient is being managed with tapering doses of corticosteroids for the last nine months with good improvement.
Subcutaneous panniculitis-like; T-cell lymphoma; rimming of fat cells
Mesenteric panniculitis (MP) is a rare disease occasionally complicated with lymphoma. A 55-year old female presented with MP accompanied by malignant lymphoma. This patient was first treated for follicular lymphoma and subsequently for panniculitis. After 6 courses of R-CHOP chemotherapy, the treatment response was partial. An additional course of salvage chemotherapy led to a complete response. Since the mesenteric mass progressed simultaneously with the regression of other lymphoma lesions, we performed a biopsy of the mesenteric mass and pathologically confirmed an MP lesion without lymphoma. Subsequent high-dose chemotherapy led to CR and the MP lesion remained stable. In the present case, MP progressed with chemotherapy. We concluded that mesenteric lesions suspected of progressing or recurring should be diagnosed pathologically even if asymptomatic.
follicular lymphoma; mesenteric panniculitis; autologous peripheral blood stem cell transplantation.
Interleukin-2 (IL-2) is now registered for the treatment of renal cell carcinoma in a number of European countries. The subcutaneous (sc) route of administration is being used increasingly because of its better toxicity profile compared with higher dose intravenous (iv) protocols. We report here a patient who developed a lobular panniculitis at the site of sc IL-2 injection which prevents continuation of sc therapy. Subsequent administration of the same IL-2 dose by iv injection caused recurrence of the problem again necessitating discontinuation of IL-2 treatment.
An elderly woman receiving long term treatment with prednisone and azathioprine for cryptogenic chronic active hepatitis developed granulomatous lipophagic panniculitis and temporal arteritis. The lymphoplasmahistiocytic inflammatory reaction pattern is common to this patient's three diseases. It is suggested that an aberration of the defence mechanisms, immunological or otherwise, is responsible for this unusual occurrence. The triple association of chronic active hepatitis, granulomatous panniculitis and temporal arteritis has not been reported previously.
Pancreatic panniculitis is a rare disease in which necrosis of fat in the panniculus and other distant foci occurs in the setting of pancreatic diseases; these diseases include acute and chronic pancreatitis, pancreatic carcinoma, pseudocyst, and other pancreatic diseases. This malady is manifested as tender erythematous nodules on the legs, buttock, or trunk. Histopathologically, it shows the pathognomonic findings of focal subcutaneous fat necrosis and ghost-like anucleated cells with a thick shadowy wall. We herein report a case of fatal pancreatic panniculitis that was associated with acute pancreatitis in a 50-yr-old man. He presented with a 3-week history of multiple tender skin nodules, abdominal pain and distension. Laboratory and radiologic findings revealed acute pancreatitis, and skin biopsy showed pancreatic panniculitis. Despite intensive medical care, he died of multi-organ failure 3 weeks after presentation.
Pancreatitis; Fat Necrosis; Panniculitis
Panniculitis is a recognized but unusual complication of a severe deficiency of α1-antitrypsin (AAT), with fewer than 100 cases described to date. Like the pathogenesis of emphysema in severe PiZZ deficiency of AAT, panniculitis has been hypothesized to be an inflammatory process, possibly related to Z AAT polymer formation and to an unopposed anti-inflammatory screen in the context of deficient serum levels of AAT. The current report presents a 31-year-old woman with PiZZ AAT deficiency-associated panniculitis. Our case extends current knowledge of AAT-associated panniculitis in 2 ways: (1) we demonstrate Z-type AAT polymers in the skin, which supports the inflammatory pathogenesis of panniculitis and the potential pro-inflammatory role of polymers; (2) we show that a high dose and long-term use of intravenous augmentation therapy (90 mg/kg body weight once weekly during 3 years) can ameliorate the frequency and severity of panniculitis associated with AAT deficiency.
α1-Antitrypsin; Polymers; Panniculitis; Inflammation; Augmentation therapy
Kimura's disease is a rare, benign, slow growing chronic inflammatory swelling with a predilection for the head and neck region and almost always with peripheral blood eosinophilia and elevated serum IgE levels. Here, we report a 25-year-old male patient with asthma, Reynaud phenomenon, eosinophilic panniculitis, bilateral inguinal lymphadenopathy and peripheral blood eosinophilia.
He responded initially to oral prednisolone with the subsidence of peripheral blood eosinophilia, asthma and the Reynaud phenomenon. But with tapering of prednisolone symptoms reappeared and hereby he was treated with cyclosporine. He has been symptom free for 6 months of follow up while taking cyclosporine 25 mg orally per day. Eosinophilia has resolved. This case shows that in addition to previously reported associations, Kimura disease may be associated with eosinophilic panniculitis and that cyclosporine could be effective in its treatment.
Several reports have been published regarding the use of cyclosporine (CSA) in the treatment of idiopathic thrombotic thrombocytopenic purpura (TTP). We hypothesized that prophylactic CSA therapy may prevent recurrences in patients with a history of multiple relapses of TTP. Nineteen patients with idiopathic TTP were enrolled on prospective studies at Ohio State University between September 2003 and May 2007. Patients achieving remission remained on CSA therapy for 6 months, allowing us to evaluate the efficacy of CSA as prophylactic therapy. CSA was administered orally at a dose of 2–3 mg/kg in a twice a day divided dose in all patients and continued for a total of 6 months. Long-term clinical follow-up with serial analysis of ADAMTS13 biomarkers during and after CSA therapy were performed to evaluate the efficacy of CSA as a prophylactic therapy. 17/19(89%) patients completed 6 months of CSA therapy in a continuous remission. Two patients relapsed during therapy with CSA and 7 patients relapsed after discontinuing CSA therapy. Ten patients have maintained a continuous remission a median of 21 months (range, 5 to 46) after discontinuing CSA. The ADAMTS13 data suggest that CSA resulted in a significant increase in the ADAMTS13 activity during therapy with CSA. 8/9(89%) relapsing patients had severely deficient ADAMTS13 activity (< 5%) suggesting this is a significant risk factor for relapse of TTP. These data support the hypothesis that prophylactic CSA improves the ADAMTS13 activity and may be effective at preventing relapses in patients at risk for recurrences of TTP.
thrombotic thrombocytopenic purpura; ADAMTS13; cyclosporine; relapse; prophylactic therapy
Objective: To describe the treatment of polymyositis (PM) and dermatomyositis (DM) with prednisone (PRED) and cyclosporin A (CSA) alone or associated with intravenous immunoglobulin (IVIg) and plasmapheresis (PEX).
Methods: Between 1992 and 1999 CSA and PRED were used to treat 20 patients with idiopathic myositis (12 with DM, eight with PM), diagnosed according to the Bohan and Peter criteria. In patients with refractory or relapsed disease, IVIg was added alone (seven cases) or synchronised with PEX (six cases). A standardised protocol was used to evaluate the patients, and assess disease activity and treatment response.
Results: Despite a transient response to PRED and CSA in 16/20 cases, this combination did not induce full remission in 13/20 cases, which led to the IVIg trial with or without PEX. Patients receiving PRED and CSA plus IVIg had a significantly higher probability of maintaining complete remission at the end of the four year follow up period than those treated with PRED and CSA alone (p<0.001). No further benefit was added by the PEX. The presence of arthritis significantly correlated with a poorer response to treatment (p<0.05). Adverse effects were gingival hyperplasia (one patient) and transient renal dysfunction (one).
Conclusions: This open study suggests that combined treatment with PRED, CSA, and IVIg is useful in patients with myositis, even those with refractory or relapsed disease; no increase in the number or type of side effects is seen.
Relapsed histologically aggressive non-Hodgkin’s lymphoma (NHL) has a poor prognosis; relapsed patients who respond to second-line chemotherapy have a better outcome after BMT, while those who do not respond to second line or are unfit for BMT have a worse prognosis and new treatments are needed. Angiogenesis is increased in aggressive NHL and could be targeted by selective cyclooxygenase-2 inhibition and metronomic chemotherapy.
Aim of the study:
Assessment of the toxicity of metronomic chemotherapy and the response and progression-free survival of relapsed diffuse large B cell lymphoma (DLCBL) patients.
Patients and methods:
Forty patients with the diagnosis of relapsed and/or refractory DLCBL. Patients included in this study may have received any number of preceding therapies (as long as one had included an anthracycline) and were not candidates for BMT. They received cyclophosphamide tab (50 mg p.o. q.d), methotrexate tab (2.5 mg p.o four times per week) and high-dose celecoxib tab (400 mg p.o. b.i.d.) until disease progression or toxicity.
All of the 40 patients included (median age, 56 years) were evaluable for response, 52% had a high international prognostic index at relapse, with a median follow-up of 8.4 months (range 4–23 months), 32.5% had a partial response and 50% has stable disease. Progression-free survival was 12 months. The median response duration was nine months. Treatment protocol was well tolerated with no major toxicities. The most common toxicity was fatigue (57.5%), myelo-suppression and gastrointestinal side effects.
Low-dose cyclophosphamide, methotrexate and high-dose celecoxib are well tolerated and active in pre-treated diffuse large cell B lymphoma. Although thrombotic events were not observed during this study, close surveillance for arterial and venous thrombotic events is recommended.
Mesenteric panniculitis (also known as sclerosing mesenteritis) is a chronic inflammatory disease of the mesenteric connective tissue. It is known to have a wide spectrum of clinical and radiological presentations. In general, biopsy is recommended for diagnosis; however, a recent study proposed that a negative positron emission tomography- computerized tomography (PET-CT) scan is accurate in differentiating benign and neoplastic mesenteric processes [Br J Radiol 2006;79:37–43]. The following case report questions the accuracy of PET-CT in this setting and confirms the requirement for biopsy to rule out the presence of mesenteric lymphoma.
Mesenteric panniculitis; Positron emission tomography; Mesentery; Mass; Lymphoma; Biopsy
Eosinophilic panniculitis is an unusual type of panniculitis characterized by a prominent infiltration of subcutaneous fat with eosinophils without an exact etiopathogenesis. To the best of our knowledge, up to now eosinophilic panniculitis has been described in only one previous case with human immunodeficiency virus disease in the literature.
Here we report the case of a 44-year-old Caucasian man, who is human immunodeficiency virus positive, diagnosed with eosinophilic panniculitis. A dermatological examination revealed multiple, confluent Kaposi’s sarcoma-like purple colored, deep plaques and nodules on his right gluteal area and right thigh. The presence of the mixed inflammatory infiltrate of lymphocytes, macrophages, and numerous eosinophils involving both septa and lobules of the subcutis were noted on the histopathological examination. On the basis of all these clinical and histopathological findings the patient was diagnosed with eosinophilic panniculitis. He was given intravenous 60mg/day methylprednisolone for 3 consecutive days a week for 6 months. The lesions resolved almost completely after 6 months.
The predominance of T helper-2 subset of T helper cells and the consequential increase in interleukin-5 cytokines accompanying peripheral eosinophilia and high serum immunoglobulin E levels may all be blamed for the development of eosinophilic panniculitis in our case study. As a result, we aim to emphasize that eosinophilic panniculitis should be kept in mind in the differential diagnosis of subcutaneous nodular lesions in patients who are human immunodeficiency virus positive. We also focus on the requirement of histopathological examination for the definitive diagnosis because the clinical features of eosinophilic panniculitis may easily be confused with Kaposi’s sarcoma.
Eosinophilic panniculitis; Human immunodeficiency virus; Kaposi’s sarcoma; Panniculitis
Pancreatic panniculitis is a rare type of disorder associated with pancreatic diseases. We describe here a case of 54-year-old man who was admitted to the Department of Dermatology with the diagnosis of erythema nodosum. The patient presented with a 9-month history of painful erythematous nodules on the extremities, joint pain and swelling, and weight loss. A highly elevated level of pancreatic lipase was found on the laboratory examinations. The biopsy specimens from the skin lesions showed subcutaneous fat necrosis. Abdominal computed tomography (CT) revealed a large mass with central necrosis in the body and tail of the pancreas. Distal pancreatectomy, splenectomy and partial transverse colectomy were successfully performed on day 17 of the hospitalization. The histopathologic findings supported the diagnosis of acinar cell carcinoma of the pancreas (ACCP). Postoperatively, the level of serum lipase returned to normal, and the skin lesions and joint manifestations gradually regressed. However, the swelling did not significantly resolve in the left knee. In view of the non-specific clinical presentation of this disease, clinicians should be alert and have a high index of suspicion for pancreatic panniculitis.
Acinar cell carcinoma of pancreas; Erythema nodosum; Pancreatic panniculitis; Subcutaneous fat necrosis
Thirty children with acute lymphoblastic leukemia had a recurrence in the bone marrow after treatment was stopped electively. A second haematological remission was achieved in 27 (90%), and the median duration of remission was shortest (six months) in those relapsing within six months of stopping treatment. Four of six children relapsing over one year after stopping treatment remained in second haematological remission. Leukaemic infiltration of the central nervous system developed in four patients remaining in marrow remission. It is concluded that conventional chemotherapy is unlikely to be effective in children with acute lymphoblastic leukaemia who relapse soon after stopping treatment, that "reprophylaxis" of the central nervous system probably with long-term intrathecal chemotherapy is essential, and that some patients relapsing after prolonged unmaintained remission may achieve long-term leukaemia-free survival.