Dermatomyositis-Like Multicentric Reticulohistiocytosis: A Clinical Clue to Verify Underlying Malignancy

2020 ◽  
Vol 4 (1) ◽  
pp. 01-04
Author(s):  
Valentina Broshtilova
Keyword(s):  
Ca 125 ◽  
Caucasian Woman ◽  
Proximal Muscle ◽  
Multicentric Reticulohistiocytosis ◽  
Underlying Malignancy ◽  
History Of ◽  
Clinical Clue ◽  
Skin Nodules ◽  
Magnetic Resonance Investigation ◽  
Clinical Subtype

Herein, an otherwise healthy 63-year-old Caucasian woman with a three month-history of progressive symmetrical swelling and of the fingers, lumbal and abdominal pain, proximal muscle weakness, and heliotropic rash resembling V- neck, histologically verified as multicentric reticulohistiocytosis (MRH), is presented. Multiple computer tomography scans failed to detect ovarian cancer despite elevated Ca 125, however, it was verified six months later by magnetic resonance investigation. Multicentric reticulohistocytosis (MRH) is a rare systematic disease that targets skin, mucoses and synovium, resulting in skin nodules, mucosal lesions and deforming painful mutilating polyarthritis. Twenty – five percent of all patients are associated with internal malignancies, hence, MRH paraneoplastic nature remains extremely controversial. Our case showed a peculiar dermatomyositis-like clinical subtype, which we consider suggestive for verification of an underlying malignancy.

scholarly journals Tinnitus, Enophthalmos, And Transient Thrombocytopenia As Symptoms Of Metastatic Breast Cancer Without Local Disease

1970 ◽  
Vol 11 (1) ◽  
Author(s):  
Gianni D’Egidio H.BSc, M.D., M.Eng ◽  
Kwadwo Kyeremanteng MD MHA
Keyword(s):  
Bone Marrow ◽  
Lobular Carcinoma ◽  
Metastatic Breast ◽  
Unknown Origin ◽  
Ca 125 ◽  
Carcinoma Of The Breast ◽  
History Of ◽  
Skin Nodules ◽  
One Year ◽  
Adenocarcinoma Of Unknown Origin

A 57 year old woman with a past medical history of oculopharyngeal muscular dystrophy presents with tinnitus, thrombocytopenia, and enophthalmos. She then develops subcutaneous skin nodules on her abdomen. Initial biopsies of the cutaneous nodules revealed adenocarcinoma of unknown origin. CT imaging of her chest, abdomen, and pelvis reveal atypical peritoneal carcinomatosis and possible diffuse bone marrow infiltration. CA -125, CEA and CA-19 are negative. Upper and lower endoscopies are normal along with bilateral mammograms of the breast. Repeat biopsy of the skin nodules and bone marrow biopsy reveals lobular carcinoma of the breast. She is treated with hormone modifying therapy for approximately one year but subsequently passes away.

030 A rapidly progressive dermatomyositis with fatality – lessons to learn

2018 ◽  
Vol 89 (6) ◽  
pp. A13.1-A13
Author(s):  
Fariha Islam ◽  
Abhishek Malhotra ◽  
Anish Sachdev
Keyword(s):  
Muscle Weakness ◽  
Positive Family History ◽  
Muscle Disease ◽  
High Dose ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Course Of Disease ◽  
Underlying Malignancy ◽  
Life Threatening ◽  
History Of

IntroductionWe report a case of dermatomyositis in a 43 year old male who presented with transient mild proximal muscle weakness but died from rapidly progressive pulmonary disease within 3 months of diagnosis.CaseThe patient presented with proximal muscle weakness, arthralgia and classic dermatomyositis rashes. He had a history of Huntington’s disease mutation with a positive family history. He had raised creatine kinase (CK) and electromyography showed myopathic changes. The diagnosis was confirmed by skin and muscle biopsy and positive anti-myositis antibodies; PM-Scl(75), MDA5 and SRP. Screening for underlying malignancy was negative but a CT chest scan showed bilateral areas of reverse halo-opacity suggestive of interstitial lung disease (ILD) although he had no respiratory symptoms at the time. He had input from dermatology, rheumatology and respiratory teams and commenced on prednisone 1 mg/kg with pneumocystis prophylaxis. He underwent bronchoscopy and broncho-alveolar lavage that did not isolate any organisms and cell differential was normal. His muscle weakness had resolved even prior to starting steroids and his CK normalised on high dose prednisolone but he developed progressive shortness of breath that led to a second admission within two months. Repeat CT chest showed extensive bilateral infiltrative disease and pneumo-mediastinum. No infective cause was found. He deteriorated rapidly despite intubation, broad-spectrum antibiotics, steroids and extracorporeal membrane oxygenation. He died whilst being considered for lung transplantation.ConclusionThis case provides valuable lessons. Even in cases of mild muscle disease, the extra-muscular involvement can be severe and life threatening in dermatomyositis. Anti-myositis antibodies can be helpful in predicting the course of disease including extra-muscular involvement. The anti-MDA5 antibodies are associated with amyopathic dermatomyositis associated with a rapidly progressive ILD and often with pneumo-mediastinum and has a poor prognosis as in our case. One should consider more aggressive therapy for these patients from the outset.

scholarly journals How Fear of COVID-19 Can Affect Treatment Choices for Anaplastic Large Cell Lymphomas ALK+ Therapy: A Case Report

Healthcare ◽  
2021 ◽  
Vol 9 (2) ◽  
pp. 135
Author(s):  
Antonello Sica ◽  
Caterina Sagnelli ◽  
Beniamino Casale ◽  
Gino Svanera ◽  
Massimiliano Creta ◽  
...  
Keyword(s):  
Cell Lymphoma ◽  
Large Cell ◽  
Brentuximab Vedotin ◽  
Caucasian Woman ◽  
Hematopoietic Stem ◽  
Drug Regulatory Agency ◽  
Clinical Center ◽  
History Of ◽  
Chemotherapy Patients ◽  
Pet Scans

Background: The t (2; 5) chromosomal rearrangement of the ALK gene with nucleophosmin 1 gene (NPM1), resulting in an NPM1–ALK fusion, was first demonstrated in 1994 in anaplastic large cell lymphoma, (ALCL), a T-cell lymphoma responsive to cyclophosphamide, abriblastine, vincristine and prednisone in approximately 80% of cases; refractory cases usually respond favorably to brentuximab vedotin. These treatments are regarded as a bridge to allogeneic hematopoietic stem cell transplantation (allo-SCT). Nowadays, transplant procedures and the monitoring of chemotherapy patients proceed very slowly because the SARS-CoV-2 pandemic has heavily clogged the hospitals in all countries. Results: A 40-year-old Caucasian woman was first seen at our clinical center in June 2020. She had ALCL ALK+, a history of failure to two previous therapeutic lines and was in complete remission after 12 courses of brentuximab, still pending allo-SCT after two failed donor selections. Facing a new therapeutic failure, we requested and obtained authorization from the Italian drug regulatory agency to administer 250 mg of crizotinib twice a day, a drug incomprehensibly not registered for ALCL ALK +. Conclusions: The response to crizotinib was optimal since no adverse event occurred, and CT-PET scans persisted negative; this drug has proved to be a valid bridge to allo-SCT.

Clinical and diagnostic aspects of deep infiltrative endometriosis (diagnosis, treatment, recurrence)

GYNECOLOGY ◽  
2021 ◽  
Vol 23 (4) ◽  
pp. 307-313
Author(s):  
Shakhnoza K. Muftaidinova ◽  
Leonid Z. Faizullin ◽  
Vladimir D. Chuprynin ◽  
Nikolai S. Ruseikin ◽  
Tatiana I. Smolnova ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Hormonal Therapy ◽  
Serum Levels ◽  
Ca 125 ◽  
Prior History ◽  
Deep Endometriosis ◽  
Preoperative Serum ◽  
Peritoneal Endometriosis ◽  
History Of ◽  
Deep Infiltrative Endometriosis

Aim. To analyze the recurrence of endometriosis after surgical treatment of patients with deep endometriosis. Materials and methods. The case histories of 90 patients aged 19 to 45 years were retrospectively analyzed. The study group consisted of 70 endometriosis patients: 20 with peritoneal endometriosis and 50 with deep infiltrative endometriosis (DIЕ). The comparison group included 20 women without endometriosis. There was an in-depth study of anamnestic data in the cohort of patients under study. The results of preoperative laboratory tests, including serum levels of the CA-125 and CA 19-9 oncomarkers, were processed. Results. Analysis of the obtained data showed that about half of the DIE patients (54%) in the main group had a history of surgical interventions for endometriosis. The number of operations was significantly higher in patients compared to the peritoneal endometriosis group (68% vs 20%, respectively; р=0.0012). Two subgroups were formed from the group of women with DIE: patients who had no history of previous surgical treatment for endometriosis and those hospitalized for repeated surgical treatment of endometriosis (patients with recurrent endometriosis). Patients with recurrent endometriosis had a significantly higher incidence of heavy menstruation, pregnancy terminations (abortions), and a high proportion of gastrointestinal diseases. Analysis of the hormonal therapy received in patients with DIE showed that every second patient with relapses (18/53%) after surgical treatment and every third patient without a prior history of surgery (5/31%) received hormonal therapy. Examination of the preoperative serum levels of CA-125 and CA 19-9 serum markers in patients with DIE showed an increase in their serum levels and a correlation with the frequency of endometriosis recurrence and the size of DIE foci. Conclusion. Despite the conservative and surgical treatment of DIE patients, the recurrence rate is still high. At present, there is no satisfactory therapy for all endometriosis patients. Therefore, the development of therapy for the conservative treatment of the disease remains an urgent task.

scholarly journals Neuroleptic malignant syndrome: a case responding to electroconvulsive therapy plus bupropion

2018 ◽  
Author(s):  
Quintí Foguet-Boreu ◽  
Montse Coll-Negre ◽  
Montse Serra-Millàs ◽  
Miquel Cavalleria-Verdaguer
Keyword(s):  
Neuroleptic Malignant Syndrome ◽  
Electroconvulsive Therapy ◽  
Potential Role ◽  
Caucasian Woman ◽  
Concomitant Treatment ◽  
Pharmacological Treatments ◽  
Severe Motor ◽  
Autonomic Instability ◽  
History Of ◽  
Psychotic Features

Neuroleptic malignant syndrome (NMS) is a severe motor syndrome occurring as a consequence of neuroleptic treatment. We present a case of a 67-year-old Caucasian woman with a history of a major depressive disorder with psychotic features. During her third hospital admission, symptoms of autonomic instability, hyperpyrexia, severe extrapyramidal side effects, and delirium appeared, suggesting NMS due to concomitant treatment with risperidone and quetiapine, among other drugs. Despite several consecutive pharmacological treatments (lorazepam, bromocriptine and amantadine) and prompt initiation of electroconvulsive therapy (ECT), clinical improvement was observed only after combining bupropion with ECT. The symptoms that had motivated the admission gradually remitted and the patient was discharged home. Bupropion increases dopaminergic activity in both the nucleus accumbens and the prefrontal cortex. Therefore, from a physiopathological standpoint, bupropion has a potential role in treating NMS. However, there is scarce evidence supporting this approach and therefore future cases should be carefully considered.

scholarly journals Exclusive Phlebosclerosis of Submucosal Veins Leading to Ischemic Necrosis and Perforation of the Large Bowel: First European Case

2018 ◽  
Vol 12 (1) ◽  
pp. 137-142 ◽  
Author(s):  
Sebastian Klein ◽  
Denise Buchner ◽  
De-hua Chang ◽  
Reinhard Büttner ◽  
Uta Drebber ◽  
...  
Keyword(s):  
Wall Thickening ◽  
Caucasian Woman ◽  
Ischemic Necrosis ◽  
Colonic Wall ◽  
Asian Patients ◽  
Life Threatening ◽  
History Of ◽  
Mesenteric Vessels ◽  
The Right ◽  
European Woman

Phlebosclerotic colitis (PC) is a rare, potentially life-threatening disease of unclear pathogenesis almost exclusively reported in Asian patients of both genders. A fibrous degeneration of venous walls leads to threadlike calcifications along mesenteric vessels and colonic wall thickening, detectable by CT. This causes disturbed blood drainage and hemorrhagic infarction of the right-sided colonic wall. This is a report of PC in a Caucasian woman in Europe without Asian background and no history of herbal medications, a suspected cause in Asian patients. CT revealed no calcification of the mesenteric vein or its tributaries. Instead, submucosal veins of the left-sided colonic wall were calcified, leading to subsequent transmural necrosis. Clinically, the patient developed a paralytic ileus and sigmoidal perforation during a 2-week hospitalization due to a bleeding cerebral vascular aneurysm. This case of a European woman with PC is unique in its course as well as its radiologic, clinical, and pathologic presentation.

scholarly journals Clinical Characteristics of Multicentric Reticulohistiocytosis and Distinguished Features from Rheumatoid Arthritis: A Single-Centre Experience in China

2021 ◽  
Author(s):  
Xiao-juan Zou ◽  
Lin Qiao ◽  
Feng Li ◽  
Hua Chen ◽  
Yun-jiao Yang ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Disease ◽  
Clinical Manifestations ◽  
Metacarpophalangeal Joint ◽  
Skin Lesions ◽  
Multicentric Reticulohistiocytosis ◽  
Specific Distribution ◽  
Pathologic Features ◽  
Skin Nodules ◽  
Distal Interphalangeal

Abstract ObjectiveTo investigate the clinical features of multicentric reticulohistiocytosis (MRH). MethodsThe clinical manifestations, laboratory examination results and histologic characteristics of eleven patients with MRH were collected and compared with those of 33 patients with rheumatoid arthritis. ResultsIn total, 72.7% of the MRH patients were women. The median age was 46 years (range: 33-84 years). Diagnosed by specific pathologic features, all MRH patients exhibited cutaneous involvement. The dorsa of the hands, arms, face and auricle were the most commonly affected areas. Nodules were also located on the legs, scalp, trunk, neck, and even the hypoglossis and buccal mucosa. Ten MRH patients (90.9%) had symmetric polyarthritis. Compared with rheumatoid arthritis (RA) patients, MRH patients were more likely to have distal interphalangeal joint (DIP) involvement (63.6% vs 24.2%, P=0.017) and less likely to have elbow (36.4% vs 72.7%, P=0.003), ankle (45.5% vs 93.9%, P<0.001) and metacarpophalangeal joint (MCP) (36.4% vs 78.8%, P=0.009) involvement. The positivity for rheumatoid factor (RF) (36.4% vs 84.6%, P=0.001) and anti-CCP antibody (9.1% vs 81.8%, P=0.000), as well as the median RF titer [43.8 (31.7-61.0) vs 175.4 (21.3-940.3), P = 0.021], in MRH patients was lower than that in RA patients. Elevation of the erythrocyte sedimentation rate (ESR) was also less common in MRH patients than in RA patients (36.4% vs 72.7%, P=0.030). After treatment with median- to large-dose corticosteroids and disease-modifying antirheumatic drugs, 8 patients achieved complete remission, and 2 patients achieved partial remission (skin lesions ameliorated, joint lesions not ameliorated). ConclusionAlways pathologically diagnosed, MRH is a systemic disease involving RA-like erosive polyarthritis and a specific distribution of skin nodules characterized by "coral beads". More DIP involvement and less elbow, ankle and MCP involvement are seen in MRH than in RA. In addition, less positive and lower-titer RF, uncommon presence of anti-CCP antibodies and ESR elevation may be helpful to distinguish MRH from RA.

scholarly journals Complications after Intrathecal Drug Delivery Due to the Underlying Malignancy in Two Patients with Intractable Cancer Pain

2013 ◽  
Vol 2;16 (2;3) ◽  
pp. E107-E111
Author(s):  
Thomas Chai
Keyword(s):  
Drug Delivery ◽  
Side Effects ◽  
Cancer Pain ◽  
Systemic Absorption ◽  
Pain Patient ◽  
Drug Side Effects ◽  
Intrathecal Drug Delivery ◽  
Underlying Malignancy ◽  
History Of ◽  
Intractable Cancer Pain

Intrathecal drug delivery is a mode of analgesic delivery that can be considered in those experiencing both refractory pain and excessive side effects from opioid and adjuvant analgesic use. Delivery of analgesic agents directly to the cerebral spinal fluid allows binding of the drug to receptors at the spinal level. Therefore, a reduced analgesic dosage can be afforded, resulting in reduction of drug side effects due to decreased systemic absorption. Drug delivery into the intrathecal space provides this benefit, yet it does not eliminate the possibility of drug side effects or risks of complications. Complications from this route of administration may be seen in the perioperative period or beyond, including infection, inflammatory mass, bleeding, and catheter or pump dysfunction, among others. This may manifest as new/worsening pain or as a neurologic deficit, such as a sensorimotor change and bladder/bowel dysfunction. Urgent evaluation with a detailed physical examination, device interrogation, and other workup including imaging is called for if symptoms suspicious for device-related problems arise. For the cancer pain patient, the underlying malignancy should also be considered as a potential cause for these new symptoms after intrathecal system implantation. We present 2 such cases of complications in the cancer pain patient after intrathecal drug delivery due to progression of the underlying malignant process rather than to surgical or device-related problems. The first patient had a history of metastatic osteosarcoma who, shortly after undergoing an intrathecal drug delivery trial with external pump, presented with new symptoms of both pain and neurologic changes. The second patient with a history of chondrosarcoma developed new symptoms of pain and sensorimotor change several days after intrathecal drug delivery system implantation. Key words: Intrathecal analgesia, intrathecal drug delivery, perioperative complications, cancer pain, malignant pain, pain pump

scholarly journals SAT-509 Sudden Onset of Malabsorption

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Gabriela Mroueh ◽  
James K Burks
Keyword(s):  
Medication Adherence ◽  
The Elderly ◽  
Sudden Onset ◽  
Primary Hypothyroidism ◽  
Caucasian Woman ◽  
Thyroid Function Tests ◽  
Autoimmune Hypothyroidism ◽  
High Clinical Suspicion ◽  
Immune Mediated ◽  
History Of

Abstract Celiac disease (CD) is an immune-mediated enteropathy caused by a reaction to gliadin which responds to a restriction to dietary gluten. It has been traditionally recognized in children and young adults, although, recently, detection in the elderly population has increased. CD occurs in 2–5% of patients with autoimmune hypothyroidism, and is more prevalent in this group than in the general population An 82-year-old Caucasian woman with primary hypothyroidism and a BMI of 16 is referred to our endocrinology clinic for help with the management of hypothyroidism. She had a history of well controlled hypothyroidism on weight-dosed levothyroxine for many years until several months prior when she developed sudden onset of diarrhea and weight loss. Since then, her thyroid function tests showed an elevated TSH despite medication adherence. Her levothyroxine dose was steadily increased to 300 mcg daily and yet, her TSH still remained elevated. Laboratory work up was done which revealed elevated transglutaminase antibodies, suggesting the diagnosis of CD. The patient refused an endoscopy for a tissue diagnosis. Even though the patient has been diagnosed with CD, she has trouble following a gluten free diet and still has intermittent diarrhea and high levothyroxine requirements. Although lack of medication adherence is common, it is important to exclude gastric or intestinal causes of malabsorption in patients with high thyroid replacement requirements. Elderly patients often have paucity of symptoms, so high clinical suspicion is necessary to diagnose these patients.

Asthma and Pneumomediastinum

PEDIATRICS ◽  
1985 ◽  
Vol 75 (5) ◽  
pp. 990-991
Author(s):  
JACOB HEN
Keyword(s):  
Physical Examination ◽  
Subcutaneous Emphysema ◽  
Childhood Asthma ◽  
Previous History ◽  
Clinical Information ◽  
Mediastinal Emphysema ◽  
Respiratory Problems ◽  
The Past ◽  
History Of ◽  
Clinical Clue

To the Editor.— An important piece of clinical information to add to Sturtz's recent review of spontaneous mediastinal emphysema in children1 is that mediastinal emphysema may be the first clinical clue to the presence of childhood asthma. Over the past 4 years, we have seen four children with cough and subcutaneous emphysema in the neck and no previous history of respiratory problems or asthma. These children had hyperinflated lungs and mediastinal emphysema demonstrated on admission chest roentgenograms and physical examination revealed wheezing.

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