Monoclonal Gammopathy of Renal Significance (MGRS) - Case Series from a Tertiary Center in Kerala.

Monoclonal gammopathy of renal significance (MGRS) has gained importance because identifying the monoclonal deposit and addressing it, rather than treating renal dysfunction as the primary pathology, has salvaged the patients from progressing into end-stage renal disease. Since it affects elderly population, there could be a propensity to misdiagnose them with cardiorenal syndrome. We present four patients of MGRS diagnosed from our center. They presented with proteinuria or unexplained renal dysfunction. Three of the patients were diagnosed to have amyloidosis, of which two had lambda-type and one had kappa amyloidosis. The fourth patient had fibrillary glomerulonephritis with kappa restriction, further evaluation of which led to diagnosis of chronic lymphocytic leukemia. Absence of "M" band in protein electrophoresis and a normal bone marrow study should not stop physicians from further evaluation. Quantitative serum immunofixation electrophoresis and electron microscopic examination of renal biopsy have become a comprehensive diagnostic tool in such patients.

Normocomplementemic Urticarial Vasculitis: An Unusual Presentation.

Urticarial vasculitis (UV) is a form of cutaneous vasculitis which lasts for >24 h. Clinically, the patients present with erythema and wheals. The level of complement decides the type of UV. This is a case of a middle-aged lady, who developed vesiculobullous lesion over her leg after trekking. She was diagnosed to have normocomplementemic UV. Bullous presentation of UV is a rare scenario.

Superior vena cava syndrome due to mediastinal Gardner fibroma presenting as syncope.

Superior vena cava (SVC) syndrome refers to the clinical manifestation due to an obstruction in the SVC; resulting in decreased venous return from the head, neck and upper extremities. The obstruction can occur either due to tumour invasion of the vessel wall with associated thrombus or due to vessel wall compression by the tumour mass. The patient being reported is a young male who presented with recurrent episodes of syncope and was found to have mediastinal Gardner fibroma causing SVC syndrome. Gardner fibroma is a benign soft tissue lesion; and its occurrence in the mediastinum resulting in SVC syndrome has not been reported yet.

Acute hepatitis due to infectious mononucleosis.

The syndrome of infectious mononucleosis is commonly seen with Epstein-Barr virus (EBV) infection. It may cause acute hepatitis, which is usually self-limiting and characterised by mildly elevated liver enzymes, but rarely jaundice. The patient being reported showcases EBV infection with jaundice, which is an uncommon scenario.

Histology of Thrombectomy Specimen Reveals Cardiac Tumor Embolus in Cryptogenic Young Stroke.

A 25-year-old woman presented with acute onset of right hemiplegia and global aphasia with National Institutes of Health Stroke Scale score of 19. Computed tomography angiography demonstrated occlusion of the left proximal middle cerebral artery. She was thrombolysed with intravenous recombinant tissue plasminogen activator (0.6 mg/kg) within 3 hours of onset of symptoms and was taken up for mechanical thrombectomy. The retrieved specimen appeared pale white and soft; histopathologic examination revealed a papillary neoplasm composed of papillae with hyalinized cores lined by endothelium, consistent with papillary fibroelastoma of cardiac origin. Transesophageal echocardiography showed no abnormalities, which can be explained by complete embolization of the mass into the cerebral circulation. On follow-up after 5 months, the patient recovered with only minimal aphasia and no cardiac symptoms. Our experience with this case highlights the importance of histopathologic examination of the mechanical thrombectomy specimen, as it provided the only clue to the etiology of stroke.

Left Ventricular Mid-Cavity Hypertrophic Cardiomyopathy with Myeloproliferative Neoplasm Leading to Multiple Cerebral Emboli.

Here we present a case of 55 year old male who presented with lower respiratory tract infection and clinical findings of systolic murmur at apex and hepatosplenomegaly and later on multiple cerebral emboli which on further evaluation turned out to be myeloproliferative neoplasm associated with cardiac mass with left ventricular mid-cavity obstruction.

Isolated Actinomycotic Swelling in Right Upper Cervical Region.

Cervicofacial actinomycosis is today a rare disease in our country. Isolated neck swelling due to actinomycosis is extremely rare. A case of 52 year old man with an isolated neck swelling due to actinomycosis without any discharging sinus is reported here.

Leukoencephalopathy with intracranial calcifications and cysts in an adult: Case report and review of literature.

Leukoencephalopathy, intracranial calcifications, and cysts (LCC) is a very rare cerebral disorder, first described in 3 children in 1996. It has subsequently been reported in adults and children from Europe and America, but has not so far been reported from Asia. We report an adult patient with pathologically proven LCC from a tertiary care hospital in South India. He presented with features of ataxia and raised intracranial pressure. Magnetic resonance imaging of the brain showed multiple bilateral cerebral cystic lesions along with diffuse white matter lesions in the cerebral and cerebellar white matter, and computed tomography of brain showed multiple calcifications in the white matter and basal ganglia. A large right cerebellar cyst causing mass effect was surgically excised. Histopathologic features were consistent with earlier reports of LCC and showed Rosenthal fibers, angiomatous changes, and calcifications. Our report suggests that although it is rare, LCC has a global distribution.

Red baby syndrome a new disease due to parvovirus B-19 observed in Kerala.

Red Baby Syndrome is a new disease seen in infants and young children. Dramatic onset of clinical symptoms with high intensity, short duration and lack of similarity with other cutaneous lesions makes it distinct. Of 50 such patients studied over a period of 5 years, half were below one year of age. Abrupt onset of high fever and generalized erythema involving the entire skin, which is swollen and tender is characteristic. These children were highly irritable and had paradoxical cry when cuddled. Rapid resolution of symptoms occurred in 7-10 days with extensive desquamation. Routine investigations were normal, C-reactive protein was raised only in 10 patients. Human Parvo virus B-19 IgM antibodies were positive in 15 out of 24 patients. Real time polymerase chain reaction was positive for human parvovirus B 19 DNA in one. Histopathological changes in the skin biopsy showed post infectious vascular injury pattern.

CNS relapse in a low risk acute promyelocytic leukemia patient treated with ATRA-based regimen: is there a role for prophylactic CNS therapy in acute promyelocytic leukemia?

Though the incidence of CNS relapse in acute promyelocytic leukemia (AML-M3 FAB classification) has increased following the advent of all-trans retinoic acid (ATRA), still CNS relapse accounts for only 2-3% of all relapses in AML-M3 trated with standard ATRA plus chemotherapy regimen. We report a case of low risk AML-M3 treated with standard therapy, developing CNS relapse while on maintenance therapy with ATRA + 6-mercaptopurine (6-MP) + methotrexate (MTX).