Myeloma-associated systemic amyloidosis masquerading as NASH-associated cirrhosis and diabetic microvascular complications.

Authors describe the case of a 60-year-old diabetic man who presented with jaundice, ascites and significant weight loss over a period of 2 months. Physical examination revealed firm hepatomegaly with ascites. On evaluation, nephropathy, axonal neuropathy, carpal tunnel syndrome and decompensated cryptogenic liver disease with portal hypertension were found fitting with the diagnosis of diabetic nephropathy and neuropathy and nonalcoholic steato-hepatitis-associated cirrhosis, respectively. It was only after tissue diagnosis and serum protein electrophoresis that a definitive diagnosis of myeloma-related amyloidosis was made. This case emphasizes the fact that due to nonspecific initial presentation and multisystem involvement, a high index of suspicion and prompt use of appropriate tests including tissue diagnosis may be required to diagnose amyloid light-chain amyloidosis, which may be a rare presenting feature of myeloma. It should be differentiated from a commoner multisystem disease like diabetes and its complications.

Disseminated cryptococcosis in an apparently immunocompetent patient presenting with primary intraventricular haemorrhage.

Disseminated cryptococcosis is uncommon and almost always occurs in HIV-infected patients. However, cryptococcosis can also be found in patients of organ transplantation, in those on disease modifying agents for rheumatological conditions and in patients with underlying immunodeficiency. Cryptococcal infection may occur in an immunocompetent patient, but the pathogenic strain is usually Cryptococcus gattii, and not C. neoformans. However, disseminated disease, especially cerebral involvement in the form of primary intraventricular haemorrhage, is exceedingly rare. We report a case of disseminated cryptococcosis with cutaneous, cerebral and bone marrow involvement in an HIV-negative, apparently immunocompetent patient. Although the patient did not have the usual immunocompromising diseases, there were clinical signs possibly indicating a weakened immune system. This report highlights the need for awareness of disseminated cryptococcosis among patients with no apparent immunocompromising conditions.

A Rare Case of Multple Myeloma (Mm) Presented With Pancytopaenia in A Patient of HIV - At Very Early Age.

Non-hodgkin`s lymphoma (NHL) is the most common haematological malignancy which is seen in HIV infected patients. Among NHLs, immunoblastic lymphomas are most common and a majority of these are diffuse large B-cell lymphomas. Multiple myeloma is a disease of the elderly. It is extremely rare below 30 years of age. On the other hand, in HIV infected individuals, the average age of presentation with plasma cell disorders is 33 years, which is far younger than the average age of presentation in general population. The incidence of AIDS defining malignancies has declined markedly, whereas non-AIDS defining cancers are being increasingly diagnosed in patients with HIV infection during the HAART-era and they may be the presenting manifestations of AIDS. Multiple myeloma, however, usually is not a first presenting feature of AIDS. The usual clinical presentation of multiple myeloma is bone pain and pallor. Pancytopaenia is a rare presenting feature of multiple myeloma. Here, we are reporting a case of pancytopaenia which occurred in a 28-year-old, newly diagnosed, HIV-1 positive female, who with the help of bone marrow examination and further investigations, was subsequently diagnosed as multiple myeloma.

Pure Motor Axonal Neuropathy, Organomegaly, Impaired Glucose Tolerance, M Protein, Skin Changes, Multiple Plasmacytomas & Acute Interstitial Nephritis in Osteolytic Myeloma: Beyond POEMS!

The authors describe a case of 52-year-old male who presented with sudden onset deterioration of weakness of both lower limbs and retention of urine. He had 1 month history of gradually progressive weakness of legs. On examination, there were lower motor neuron signs in lower extremity, digital clubbing and a lump over left iliac fossa. Routine blood tests showed impaired glucose tolerance, confirmed by oral glucose tolerance test while renal parameters were normal. Magnetic resonance imaging of spine documented osteolytic lesions, long segment epidural mass in thoracic spine and a mass overlying the left iliac bone, both were revealed to be plasmacytoma following cytology. Ultrasonography of abdomen showed splenomegaly. Nerve conduction studies showed gross axonal, motor, asymmetric polyneuropathy with conduction block involving all the four extremities, mainly lower limbs with sensory sparing. Serum protein electrophoresis showed M spike, and bone marrow showed diffuse neoplastic plasma cell proliferation. Osteolytic lesion was present in skull radiograph. Then in the course of illness the patient developed acute renal failure due to acute interstitial nephritis as evidenced from proteinuria and kidney biopsy, which improved with steroids and chemotherapy but unfortunately we lost the patient after 2 weeks of initiation of chemotherapy.

An unusual cause of breast lump: isolated tuberculosis of the breast.

A 60-year-old woman presented with a painful lump of 4 months duration in her right breast. She denied any history of breast trauma or family history of breast cancer. Physical examination detected a firm, tender mass in the upper inner quadrant of her right breast without an associated sinus tract. Cytology from fine-needle aspiration of the swelling showed epithelioid cells, lymphohistocytic aggregates, and necrosis. Diagnosis was confirmed by the identification of acid-fast bacilli in tissue sections using Ziehl-Neelsen staining.

Epithelial-myoepithelial carcinoma of the maxillary sinus: a rare case.

Epithelial-myoepithelial carcinoma (EMC) is a rare tumor that commonly involves the salivary glands. EMC of the maxillary sinus is extremely rare. We describe here a case of a 50-year-old patient who presented with right cheek swelling for 7 years and bilateral nasal obstruction and right nasal bleeding for 3 months. A pinkish polypoidal mass was seen in the right nasal cavity. Computed tomography scan revealed an expansile soft tissue mass in the right maxillary sinus with erosion of all of its walls. Histopathological examination of the excised specimen confirmed myoepithelial carcinoma with positive reaction to S-100 and smooth muscle actin. Subsequently, the patient developed regional nodal metastasis in the neck for which neck dissection was performed. Histopathology of the neck dissection specimen revealed metastatic EMC. EMC is rare tumor and a diagnostic dilemma. It should be considered in cases showing dual tumor cell population with clear cell change in histopathology. Common differential diagnosis includes metastatic renal cell carcinoma, acinic cell carcinoma, adenoid cystic carcinoma, mucoepidermoid carcinoma, oncocytoma, and sebaceous carcinoma. Laryngoscope, 2012.

Manual stapedotomy still holds good in the era of laser.

CONTEXT: In the present era of stapedotomy, there is an inevitable role for laser. But the conventional technique with manual burr still has its own merits in various settings such as usage in resource poor setting in developing countries and avoidance of laser hazards. AIMS: To evaluate the audiometric outcomes after manual stapedotomy. SETTINGS AND DESIGN: The present study was retrospective record-based study. Patients who have been diagnosed otosclerosis and those who were not willing for surgery with laser, but gave consent for manual stapedotomy were included for the study. MATERIALS AND METHODS: Preoperatively, and at each subsequent post-operative follow-up visits, patients were required to undergo a pure-tone audiogram. The air-bone gaps at the end of 6 months were used for final analysis. STATISTICAL ANALYSIS USED: Data was analyzed with using Statistical Package for Social Sciences (SPSS) version 12 (Chicago, IL, USA). Descriptive frequency distributions, mean, standard deviation of audiometric data were calculated. The paired t test was done to see the improvement in the air bone gap post-operatively. RESULTS: The mean age of presentation is 32.2 years. Overall, the male:female ratio was found to be 1:1.2. Post-operative air bone closure to 15 decibels was obtained in 80% of patients. There was no significant correlation between the pre-operative hearing loss and post-operative gain, age, and gender of distribution of focus. CONCLUSIONS: The post-operative hearing after stapedotomy has been remarkable in all the patients even with manual burr. There were no major vestibular complications in any of these patients. The study has shown that the significant post-operative hearing results can be still achieved with meticulous surgery by an experienced surgeon with manual burr in the present laser era.