- Dermoscopic follow-up of therapeutic response in mantle cell lymphoma with secondary involvement of the scalp. [Journal Article]
- JCJ Cosmet Dermatol 2018 Dec 05
- The scalp is a potential location for both benign and malignant tumors. Lymphoproliferative diseases can involve the skin as a primary or secondary manifestation. Dermoscopy is a noninvasive diagnost...
The scalp is a potential location for both benign and malignant tumors. Lymphoproliferative diseases can involve the skin as a primary or secondary manifestation. Dermoscopy is a noninvasive diagnostic tool for rapid diagnosis, screening, and follow-up of the majority of skin tumors. Mantle cell lymphoma (MCL), a rare type of aggressive systemic lymphoma, usually occurs as a generalized lymphadenopathy, commonly with infiltration of the bone marrow, spleen, gastrointestinal tract, and Waldeyer's ring. In rare cases, it can also involve other structures, such as the lungs, central nervous system, liver, or skin. We report the case of a 74-year-old male patient suffering from MCL since 2015. Complete remission was obtained after R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) treatment. During maintenance therapy with rituximab, a solitary tumor occurred on the scalp. Dermoscopy of the lesion suggested relapse because of the presence of multiple chaotically distributed short linear vessels with multiple red dots within the hair follicles. Histological examination confirmed the diagnosis of MCL. After second-line therapy with rituximab and bendamustine (R-B), the tumor of the scalp completely disappeared and dermoscopy showed no abnormalities.
- Isolated Intraventricular Rosai-Dorfman Disease. [Journal Article]
- AJAsian J Neurosurg 2018 Oct-Dec; 13(4):1285-1287
- Rosai-Dorfman disease (RDD) is a benign histioproliferative disorder characterized by generalized lymphadenopathy, weakness, anemia, and rarely extranodal involvement. The disease affects the central...
Rosai-Dorfman disease (RDD) is a benign histioproliferative disorder characterized by generalized lymphadenopathy, weakness, anemia, and rarely extranodal involvement. The disease affects the central nervous system (CNS) very rarely and at a relatively older age. Isolated intracranial involvement is very rare. RDD tends to present as dural masses mimicking meningioma, though very rare case reports describe isolated parenchymal involvement in the CNS. Only one case of intraventricular RDD has been described so far to the best of our knowledge. We present a very rare presentation of this disease as an isolated intraventricular presentation in the region of the atria of the lateral ventricles.
- CD8-positive Pseudolymphoma in Lues maligna and HIV with monoclonal TCR-beta rearrangement. [Case Reports]
- JCJ Cutan Pathol 2018 Nov 08
- A 42-year-old Caucasian man suffered from disseminated plaques and ulcerated nodules for six weeks. He had weight loss and generalized lymphadenopathy. Underlying diseases were not known up till then...
A 42-year-old Caucasian man suffered from disseminated plaques and ulcerated nodules for six weeks. He had weight loss and generalized lymphadenopathy. Underlying diseases were not known up till then. Based on a skin biopsy the diagnosis of CD8-positive cutaneous T-cell lymphoma, type mycosis fungoides was made in a pathological reference center for lymphoma. A reproducible TCR-beta rearrangement was detectable. Before starting therapy, a new biopsy was taken and the previous diagnosis was re-evaluated taking clinical images and symptoms into account. Based on both, the diagnosis of a CD8+ pseudolymphoma in lues maligna and HIV was made. We highlight histopathologic clues for the correct diagnosis, and we emphasize the indispensability of clinical-pathological correlation. Furthermore, we discuss the differential diagnosis of CD8+ lymphoproliferative disorders. This article is protected by copyright. All rights reserved.
- A Case of Generalized, Superinfected Dermatitis and Inguinal Mycobacterium Lymphadenitis - TB or not TB? [Journal Article]
- ADActa Dermatovenerol Croat 2018; 26(3):270-272
- Dear Editor, Eczema is an inflammatory dermatitis mediated by cellular immunity, with an etiology in which environmental, immunological, and genetic factors are involved. Skin inflammation through pr...
Dear Editor, Eczema is an inflammatory dermatitis mediated by cellular immunity, with an etiology in which environmental, immunological, and genetic factors are involved. Skin inflammation through proinflammatory cytokines creates a favorable environment for microbial antigens and optimal conditions for infection (1). In case of underlying immunosuppression, inflammatory features of dermatitis and superimposed infections are more severe. The presence of minor trauma of the skin in the form of fissures can favor both easier inoculation of some bacterial germs, leading to a dermatitis superinfection, and/or the transcutaneous inoculation of atypical mycobacteria, with a possibility of developing localized types of tuberculous lymphadenitis (TLA). TLA, the localized type of systemic tuberculosis (TB) infection, is the most common form of extra-pulmonary TB in developing countries (2), while lymphadenitis due to atypical mycobacteria is a localized disease, more frequently seen in developed countries (3,4). In tuberculosis, the transmission of Mycobacterium tuberculosis is airborne, while in atypical mycobacterium lymphadenitis transmission can be both airborne or by ingestion or inoculation (5). In both forms of TB, lymphadenopathy evolves towards abscess and presents fibrotic scars or calcifications upon healing (6). A positive diagnosis involves a clinical and epidemiological investigation, a purified protein derivative (PPD) skin test, ultrasound, and CT / MRI of lymph node masses. A lymph node biopsy is used to confirm the diagnosis of TB and PCR, while positive culture confirms the etiology of TB lymphadenitis. The differential diagnosis of TLA is difficult: neoplastic, bacterial, or viral and fungal infections, sarcoidosis, Castleman's disease, drug reactions, etc. (5). TB-induced immunosuppression may favor the development of fungal and bacterial infections, sometimes severe and poorly responsive to treatment. On the other hand, immunosuppressive conditions increase the risk of extra-pulmonary TB (2). A 40-year old woman who had experienced recurrent episodes of dermatitis over the previous 7 years was hospitalized with fever, malaise, and a disseminated erythematous and crusted, exudative, and flexural itching rash (Figure 1). There were fetid, purulent secretions at the conjunctival, auricular, genital, and umbilical areas. The clinical exam also revealed lymphadenopathy syndrome (large, painful submandibular, cervical, and axillar bilateral lymph nodes; an indurated, painful, and adherent left inguinal lymph node of 5-6 cm). Microbial cultures isolated multiple multi-drug-resistant bacteria (SAH-MRSA, Acinetobacter baumannii, Enterococcus faecalis, E. coli, Enterobacter) and Candida albicans in the oral cavity and conjunctival, auricular, nasal, umbilical, and genital areas. The skin biopsy confirmed the diagnosis of dermatitis. PPD skin test was 21 mm. Other tests (HIV and syphilis serology, blood culture, chest X-ray) were negative. Systemic treatment with vancomycin, metronidazole, fluconazole, local antiseptic compresses, and topical corticosteroid ointments was initiated. 2 days after starting the treatment with vancomycin, Redman syndrome occurred (headache, dyspnea, colicky pains, myalgia, rush, fever (39 °C), hypotension (80/40 mmHg), and tachycardia (100 bpm)). This syndrome resolved upon discontinuation of Vancomycin. Further treatment with imipenem/cilastatinand linezolid for 14 days lead to a favorable response with amelioration of the symptoms. Biopsy of the submandibular lymph node raised the suspicion of Castleman's disease; however, due to the overall incomplete clinical picture (no night sweats, no weight reduction, lack of hepatosplenomegaly and peripheral neuropathy), we decided to perform a biopsy of an inguinal lymph node. The histopathological aspect suggested TLA (lymphoid hyperplasia predominantly diffuse, reactive, presenting tuberculous follicles with central caseous necrosis) (Figure 2). A combination of specific antituberculous drugs (isoniazid, rifampicin, pyrazinamide, and ethambutol) for 6 months resolved the lymphadenopathy syndrome with no further recurrence of eczema and skin infections. Certain delayed hypersensitivity mechanisms are involved both in dermatitis and in TB. CD4 lymphocytes are the primary mediators of anti-TB immunity, while proinflammatory cytokines mediate the activation of macrophages involved in controlling bacillary growth (1). In cases of superinfected dermatitis, microbial exotoxins penetrate the skin barrier more easily due to inflammation. Released cytokines (IL-1, TNF, and IL12) favor the expression of E-selectin on endothelial vascular growth factor and on skin lymphocyte antigen expression, with amplification of initial skin inflammation and creating favorable conditions for microbial colonization and infection (7). The common denominator in dermatitis and TB are the circulating immune complexes (up to 56% of TB cases), which are formed by the interaction between an antibody and bacterial antigen (8), which was in this case evidenced by increased levels of IgA and IgG. In our case, the frequent recurrences of infected dermatitis with multiple multi-drug-resistant germs that were poorly responsive to treatment and displayed a severe evolution towards generalization as well as the lymphadenopathy and the persistence of a biological inflammatory syndrome indicated that another immunosuppressive cause could be involved. Isolated bacterial and fungal germs changed the immune status of the patient. The risk of mycobacterium infection was increased by the environment they created and the patient's underlying skin inflammation. The diagnosis of TB lymphadenitis was established by the histopathologist, but in the absence of PCR we could not determine whether the TB infection was caused by Mycobacterium tuberculosis or by atypical mycobacteria. Given that there was no evidence of other sites of TB infection, we conjectured that inoculation of mycobacterium took place at the skin lesion and that an atypical mycobacterium might have contributed to the etiology of the TLA. In our case, the anti-tuberculous drugs and skin infection treatment with follow-up of the side-effects led to complete remission of mycobacterium lymphadenitis, dermatitis, and infectious processes, without relapses. In conclusion, in the present case chronic dermatitis alongside infection with multi-drug-resistant germs led to an immunosuppressive status which, when associated with the presence of multiple skin ports of entry, allowed a mycobacterial infection at the inguinal lymph node level. Inguinal TLA induced severe dermatitis and difficulties in diagnosis and treatment.
- T-Cell Prolymphocytic Leukemia: An Experience from a Tertiary Cancer Centre in South India. [Journal Article]
- IJInt J Hematol Oncol Stem Cell Res 2018 Apr 01; 12(2):132-135
- Background: T-cell prolymphocytic leukemia (T-PLL) is a rare lymphoid malignancy with dismal prognosis. Most patients have increased lymphocyte count (>1,00,000/dL) and widespread disease at present...
Background: T-cell prolymphocytic leukemia (T-PLL) is a rare lymphoid malignancy with dismal prognosis. Most patients have increased lymphocyte count (>1,00,000/dL) and widespread disease at presentation. Despite high response rate seen with alemtuzumab, the disease relapse is inevitable. Materials andMethods: This was a retrospective observational study done at a tertiary cancer center in South India. All patients diagnosed with T-PLL from August 2010 to July 2015 were studied for the clinical characteristics, pathological findings and treatment outcomes. Results : Seven patients were diagnosed as T-PLL over a period of 5 years. The median age at diagnosis was 51 years. In the present series, 6 patients (86%) had splenomegaly and 3 had hepatomegaly (43%). Generalized lymphadenopathy was seen in 4 (57%) patients at presentation. Skin lesions were seen in 5 (71%) patients, whereas pleural effusion was seen in only one patient (14%). All had elevated total leukocyte count, with more than 1, 00,000/dL in 4 patients. The median survival was 5 months with different chemotherapy (CT) regimens (5 patients treated with CT and 2 received best supportive care). Conclusion: T-PLL is a rare disease with no definite treatment guidelines. At present, the best outcomes are achieved if treatment with alemtuzumab is followed by stem cell transplant, but the disease invariably relapses. Countries where affordability remains a big challenge, the best approach needs to be defined beyond the monoclonal antibodies and transplant.
- [Schnitzler syndrome]. [Journal Article]
- HHautarzt 2018; 69(9):761-772
- Schnitzler syndrome is a very rare acquired systemic disease with many similarities to hereditary autoinflammatory syndromes. The main characteristics are generalized exanthema and a monoclonal gammo...
Schnitzler syndrome is a very rare acquired systemic disease with many similarities to hereditary autoinflammatory syndromes. The main characteristics are generalized exanthema and a monoclonal gammopathy with IgM. Other clinical features include fever, muscle, bone and/or joint pain, and lymphadenopathy. About 15-20% of patients with Schnitzler syndrome develop lymphoproliferative diseases and, in rare cases, amyloid A (AA) amyloidosis can occur if the disease is not treated. Activation of the innate immune system, especially interleukin(IL)-1β, is central in the pathogenesis of the disease. Consequently, complete control of disease symptoms can be achieved in 80% of patients by treatment with the IL-1 receptor antagonist anakinra.
- Lymphoproliferative disorder in an elderly rheumatoid arthritis patient after longterm oral methotrexate administration: A case report. [Journal Article]
- MCMol Clin Oncol 2018; 9(3):293-296
- Methotrexate-associated lymphoproliferative disorder (MTX-LPD) is frequently reported in the literature; however, its pathophysiology has not been fully elucidated to date. We herein describe a case ...
Methotrexate-associated lymphoproliferative disorder (MTX-LPD) is frequently reported in the literature; however, its pathophysiology has not been fully elucidated to date. We herein describe a case of MTX-LPD that occurred after long-term treatment with oral MTX in a 67-year-old Japanese woman with rheumatoid arthritis (RA) who presented with generalized lymphadenopathy of the neck. The patient had been diagnosed with RA 24 years earlier, and had been on oral MTX for 20 years. The patient noticed a mass on her neck, which prompted a visit to our hospital. The mass was confirmed as diffuse large B-cell lymphoma by biopsy. MTX treatment was discontinued, which resulted in a reduction in the size of the mass and improvement of the patient's symptoms. Therefore, clinicians must be aware of MTX-LPD as a differential diagnosis for patients with rheumatological conditions on long-term MTX therapy presenting with signs and symptoms suggestive of lymphoma.
- Current trends in opportunistic infections in children living with HIV/AIDS in a tertiary care hospital in Northern India. [Journal Article]
- IJIndian J Sex Transm Dis AIDS 2017 Jul-Dec; 38(2):142-146
- A prospective cohort study was undertaken from November 2010 to March 2012 at Kalawati Saran Children's Hospital (KSCH), Lady Hardinge Medical College (LHMC), New Delhi. The study included all HIV po...
A prospective cohort study was undertaken from November 2010 to March 2012 at Kalawati Saran Children's Hospital (KSCH), Lady Hardinge Medical College (LHMC), New Delhi. The study included all HIV positive children aged between 0-15 years that were registered in the anti-retroviral therapy (ART) centre during the study period. HIV +ve children enrolled at the ART centre were started on ART on the basis of CD4counts (National/NACO guidelines).
- Schnitzler syndrome co-occurring with idiopathic multicentric Castleman disease that responds to anti-IL-1 therapy: A case report and clue to pathophysiology. [Journal Article]
- CRCurr Res Transl Med 2018; 66(3):83-86
- Patients with HHV-8-negative/idiopathic multicentric Castleman disease (iMCD) experience systemic inflammatory symptoms and polyclonal lymphoproliferation due to an unknown etiology. Schnitzler's syn...
Patients with HHV-8-negative/idiopathic multicentric Castleman disease (iMCD) experience systemic inflammatory symptoms and polyclonal lymphoproliferation due to an unknown etiology. Schnitzler's syndrome (SS) is characterized by recurrent urticarial rash, monoclonal IgM gammopathy, and other clinical signs of inflammation. To our knowledge, we report the first case of iMCD associated with SS and the fourth case of anakinra inducing a complete response for an iMCD patient. A forty-four year old woman with a history of a recurrent urticarial rash, presented to our hospital complaining of 6 months of night sweats, fever, chronic urticaria, iliac bone pain, and generalized lymphadenopathy. An IgM Kappa monoclonal component was measured at 7.8g/L. A lymph node biopsy revealed histopathological features consistent with the plasma cell variant of iMCD. She was diagnosed with SS and iMCD. Anti-IL-1 treatment with anakinra (100mg/day) was introduced. Within 48h, we observed improvement in the fever and the urticarial rash. By one month, we considered the patient in complete remission. Two years later, the remission is persistent while the patient is still under therapy. Though this is only the fourth reported case of anakinra in iMCD, this is yet another case demonstrating the effectiveness of anti-IL-1 blockade in SS. We hypothesize that uncontrolled cytokine production is responsible for both the SS and the iMCD. The etiologies of SS and iMCD are unknown, and future research is necessary.
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- Cat-Scratch Disease in an AIDS Patient Presenting with Generalized Lymphadenopathy: An Unusual Presentation with Delayed Diagnosis. [Case Reports]
- AJAm J Case Rep 2018 Aug 02; 19:906-911
- CONCLUSIONS: Cat-scratch disease may present with prolonged generalized lymphadenopathy, an unusual presentation in HIV patients with advanced immunosuppression. Awareness of the possibility of CSD in a similar clinical scenario may prompt early recognition and management of this disease.