Duodenal Ulcer with Massive Gastrointestinal Hemorrhage as an Initial Manifestation in Multiple Myeloma with Extramedullary Disease: A Case Report

Plasma cell neoplasms are characterized by dysregulated proliferation of mature B cells, which can present with either single (solitary plasmacytoma) or systemic (multiple myeloma (MM)) involvement. MM with extramedullary plasmacytoma (EMP) is a rare disease that accounts for approximately 3–5% of all plasmacytomas. EMP with gastrointestinal (GI) system involvement is an even rarer entity, accounting for <1% of MM cases. We present a case of aggressive MM with EMP invading the duodenum, initially presented with massive upper GI hemorrhage and small bowel obstruction. A 67-year-old woman was admitted to our hospital owing to a lack of either gas or feces passage for 3 days. Abdominal distention and vomit with a high coffee ground content were observed for 24 h. The patient’s condition was initially diagnosed as small bowel obstruction, upper gastrointestinal bleeding, severe anemia, acute renal failure, and hypercalcemia. Furthermore, an analysis of immunoelectrophoresis in the blood, bone marrow aspiration, and tissue biopsy supported the diagnosis of MM and EMP invading the duodenum, upper GI hemorrhage, and small bowel obstruction. Our study provided the possible involvement of MM and EMP in the differential diagnosis of patients with unexplained GI hemorrhage and small bowel obstruction. A thorough review of the literature regarding the association between MM, GI hemorrhage, and small bowel obstruction is presented in this study.

Comparison of Clinical, Immunological And Radiological Characteristics In Autoimmune GFAP Astrocytopathy, MOGAD And AQP4-Igg +NMOSD Mimicking Infectious Meningitis As The Initial Manifestation

Objective: Several autoimmune CNS inflammatory diseases, including autoimmune glial fibrillary acidic protein astrocytopathy (A-GFAP-A), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and aquaporin-4-immunoglobulin-G-positive neuromyelitis optica spectrum disorders (AQP4-IgG+ NMOSD) often presented initially with similar infectious meningitis-like symptoms. However, it was not easy to differentiate them at disease onset without antibody detection. The present study aimed to compare the clinical, immunological and radiological features among the three diseases. Methods: In our single-center cohorts, 9 A-GFAP-A, 17 MOGAD and 11 AQP4-IgG+ NMOSD patients mimicking infectious meningitis as initial symptoms were retrospectively included. The autoantibodies were detected with cell-based assays. The clinical, immunological and radiological characteristics of the three groups were summarized. Results: AQP4-IgG+ NMOSD patients were statistically more often in men (10, 90.9%, P=0.003). Tremor was predominated in A-GFAP-A (4, 44.4%) over MOGAD (1, 5.9%, P= 0.034) and never found in AQP4-IgG+NMOSD (0, P=0.026). The Modified Rankin Score (mRS) at the clinical nadir of diseases was lower in AQP4-IgG+NMOSD (2.2 [IQR, 1-3]) compared to A-GFAP-A (3.7 [IQR, 3-5], P=0.04). On CSF examination, white blood cell count (WBC) was higher in A-GFAP-A (median, 272×106/L [range, 0-1600]) compared to AQP4-IgG+NMOSD (median, 12×106/L [range, 0-48], P=0.049). Significant increase in CSF protein (1490.7±871.2 mg/L), lactic acid (3.43±0.81 mmol/L), IgG (130.9±60.4 mg/L), IgM (8.6±6.1mg/L) and IgA (23.0±11.4mg/L) levels in A-GFAP-A was found compared to MOGAD (CSF protein: 606.7±379.4 mg/L, P<0.001; lactic acid: 2.15 ± 0.62mmol/L, P<0.001; IgG: 77.9±71.3 mg/L, P=0.043; IgM, 2.7±2.9mg/L, P=0.002; IgA, 11.3±12.1mg/L, P=0.012) and AQP4-IgG+NMOSD (CSF protein: 441.8±178.0 mg/L, P<0.001; lactic acid: 2.40 ± 0.66 mmol/L, P=0.003; IgG, 53.2±30.3 mg/L, P=0.01; IgM, 2.1±3.9mg/L, P=0.003; IgA, 5.2±5.0mg/L, P=0.001). Over half of the A-GFAP-A patients (5/8, 62.5%) showed small (<2 cm), symmetrical lesions in ganglia and thalamus (5/8, 62.5%), but never in MOGAD (0%, P=0.001) and AQP4-IgG+NMOSD (0%, P=0.026). Diffuse meningeal enhancement was common in A-GFAP-A (8, 88.9%) compared to MOGAD (5, 29.4%, P=0.011) and AQP4-IgG+NMOSD (1/6, 16.7%, P=0.011). Acute disseminated encephalomyelitis (ADEM) -like lesions occurred frequently in MOGAD (6/16, 37.5%) but never in A-GFAP-A and AQP4-IgG+NMOSD (P=0.02). Conclusion: Our study demonstrated that several signs including the symptom of tremor, a more severe disease course, higher CSF immunological profiles and ganglia bilateral symmetrical lesions, diffuse meningeal enhancement were distinct features in A-GFAP-A, and ADEM-like lesions occurred only in MOGAD mimicking infectious meningitis as initial symptoms, providing possible clinical implications for patient differential diagnosis.

Syncope as the Initial Manifestation of Advanced Nasopharyngeal Carcinoma: A Case Report

Carotid sinus syndrome is a principal cause of syncope in the elderly. Syncope, associated with carotid sinus syndrome which is secondary to metastasis of advanced nasopharyngeal carcinoma, rarely occurs. The current study reported a 66-year-old woman, who presented with a history of frequent and recurrent syncope as the initial symptom, and was eventually diagnosed with advanced nasopharyngeal carcinoma. The positron emission tomography scan demonstrated a diagnosis of advanced nasopharyngeal carcinoma with involvement in carotid sheath space, and nasopharyngeal biopsy revealed non-keratinized nasopharyngeal carcinoma. After diagnosis and treatment, the patient had no recurrence of syncope. In summary, our case study suggests that great importance should be attached to potential intrinsic causes of syncope especially in the case of nasopharyngeal carcinoma, as it is an insidious malignancy which needs to be precisely identified.

Magnetic resonance findings may aid in diagnosis of protracted febrile myalgia syndrome: a retrospective, multicenter study

Background Protracted febrile myalgia syndrome (PFMS) is a rare complication of Familial Mediterranean fever (FMF). The diagnosis is based on clinical symptoms and is often challenging, especially when PFMS is the initial manifestation of FMF. The aim of this report was to describe the magnetic resonance imaging (MRI) findings in pediatric patients with PFMS. Results There were three girls and two boys ranging in age from 6 months to 16 years, all of Mediterranean ancestry. Three had high-grade fever, and all had elevated inflammatory markers. MRI of the extremities yielded findings suggestive of myositis, which together with the clinical picture, normal CPK levels, and supporting family history of FMF, suggested the diagnosis of PFMS. Out of most common MEFV mutations tested, one patient was homozygous for M694V mutation, three were heterozygous for M694V mutation, and one was compound heterozygous for the M694V and V726A mutations. Conclusions MRI may serve as an auxiliary diagnostic tool in PFMS.

Localisation Buccale Secondaire D’un Carcinome Renal

Renal cell carcinomas are tumors known for their metastatic potential. The lungs, the lymph nodes, the lungs, the spleen, the adrenal gland and the cervix remain the metastatic sites of predisposition. The symptoms of metastatic lesion may be due to the initial manifestation of renal malignancy. We report in this work a buccal localization of metastases from renal cell carcinoma to clear cells in a patient aged 65 years or less in our department of otolaryngology and cervical-facial surgery.

Kawasaki Shock Syndrome with Initial Presentation as Neck lymphadenitis: A Case Report

Kawasaki disease (KD) is an acute systemic vasculitis of unknown cause that mainly affects infants and children and can result in coronary artery complications if left untreated. A small subset of KD patients with fever and cervical lymphadenitis has been reported as node-first-presenting KD (NFKD). This type of KD commonly affects the older pediatric population with a more intense inflammatory process. Considering its unusual initial presentation, a delay in diagnosis and treatment increases the risk of coronary artery complications. Herein, we report the case of a 9-year-old female with fever and neck mass that rapidly deteriorated to shock status. A diagnosis of KD was made after the signs and symptoms fulfilled the principal diagnostic criteria. The patient’s heart failure and blood pressure improved dramatically after a single dose of intravenous immunoglobulin. This case reminds us that NFKD could be the initial manifestation of KDSS, which is a potentially fatal condition. We review the literature to identify the overlapping characteristics of NFKD and KDSS, and to highlight the importance of early recognition of atypical KD regardless of age. We conclude that unusually high C-reactive protein, neutrophilia, and thrombocytopenia serve as supplemental laboratory indicators for early identification of KDSS in patients with NFKD.

A Review of Primary Immunodeficiency Diseases with Skin Manifestations

Introduction: Primary immunodeficiencies (PID) are rare heterogeneous disorders with defects in which one or more components of the immune system are malfunctioning. Clinical presentations of the patients according to type of immunodeficiency are variable. The majority of these patients are susceptible to infections depending on the type of disorder. In these patients, one of the most important and common symptoms is a skin manifestation that in many cases helps to diagnose the disease. Skin symptoms can include infectious-inflammatory-autoimmune-allergic manifestations and malignancies. In some cases, skin involvement can be the initial manifestation of immunodeficiency diseases, so understanding the relationship between the type of primary immunodeficiency and the type of skin involvement is very important in diagnosing the disease. The majorities of skin diseases are not pathogenomonic in primary immunodeficiencies and may be seen in other diseases with normal levels of immunity. However, there are numerous skin findings that are so characteristic of immunodeficiency diseases that it is necessary to evaluate the immune system. Conclusion: Skin is an organ that may be involved in many diseases, including primary immunodeficiency. Sometimes skin is the first organ involved in immune deficiencies. Therefore, recognizing skin manifestations in these patients is one of the most important factors in early diagnosis of these people.