A 75-year-old man, with a long history of recurrent lymphoplasmacytoid lymphoma, presented with diffuse large-cell lymphoma affecting adrenal glands and causing severe hypoadrenalism. The lymphoma responded to rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone (R-CHOP) chemotherapy. Seven months postcompletion of chemotherapy, he developed signs of gastroenteritis and septicaemia. He deteriorated 24 h postadmission with a significant fall in Glasgow Coma Scale Score. Polymerase chain reaction testing of cerebrospinal fluid suggested enteroviral encephalitis. He responded symptomatically to intravenous immunoglobulins. His immunoglobulin levels were monitored weekly and supplemented to maintain immunoglobulin G level at 10 g/l, but in spite of this, his neurological condition deteriorated and he died after 14 weeks. Rituximab can cause prolonged B-cell deficiency. We speculate that profound immunosuppression induced by rituximab, together with previous chemotherapy, predisposed this patient to fatal enteroviral meningoencephalitis.
Hyperhomocysteinemia known to be associated with increased thrombotic tendency has been considered as a risk factor for coronary artery disease, atherosclerosis, venous thrombosis, and stroke. There are three main genes MTHFR, cystathionine beta-synthase (CBS) and methionine synthase (MS) and it's genetic variant that are known to influence the homocysteine metabolism leading to hyperhomocysteinemia. There is scarcity of Indian data on hyperhomocysteinemia and genetics variants in patients with thrombosis. Hence the objective of present study was to determine MTHFR, CBS, and MS genetic variants in thrombosis patients from Indian population. Genetic variant analysis was performed on thrombosis patients to detect MTHFR C677T (rs1801133), MTHFR A1298C (rs1801131), MS A2756G (rs1805087) and CBS T833C (rs5742905) mutations. The mutant allele frequencies of MTHFR 677T, MTHFR 1298C, MS2756G and CBS 833C were observed to be 16.1%, 37.5%, 34.1% and 5.8% respectively. MTHFR 677TT genotype was observed to be significantly associated with elevated homocysteine (Hcy) levels (64.65 lmol/L) alleles as compared to CC alleles (32.43 lmol/L) and CT alleles (30.54 lmol/L). MTHFR A1298C, MS A2756G and CBS T833C genotypes did not showed significant association with higher Hcy levels. Thus, in Indian patients with thrombosis only MTHFR T677T genotype was observed to be significantly associated with hyperhomocysteinemia.
are recommended when neuraxial techniques are planned. Ultrasound-assisted spinal anaesthesia may be helpful with challenging spinal anatomy but is not always successful and real-time ultrasound-guided spinal anaesthesia may be necessary. 8 However, an experienced practitioner in ultrasound-guided spinal techniques may be required.
The anaemia in beta thalassemia major is multifactorial. It is in part due to the defect in globin chain synthesis leading a hypochromic microcytic anemia, in part due to a shortened red cell survival and in part due to ineffective erythropoiesis. Ineffective erythropoiesis is the result of alpha chain deposition in erythroblasts, which leads to apoptosis and ingestion of erythroblasts by macrophages. It is the hyperplastic ineffective erythropoiesis that is responsible for many of the pathological effects of untreated or undertreated thalassemia major.The bone marrow aspirate films illustrated are from a boy with beta thalassemia major, aged 3 years 6 months, who had been regularly transfused from the age of 6 months. He had been on chelation therapy with subcutaneous deferoxamine (desferrioxamine) 6 nights a week from the age of 18 months with the most recent serum ferritin being 1800 lg/l. Hepatic iron was 4.4 mg/g. The bone marrow aspiration was performed prior to a hemopoietic stem cell transplant.The May-Grü nwald-Giemsa-stained film (left) shows hyperplastic erythropoiesis and two macrophages containing ingested erythroblasts and cellular debris. The Perls-stained film (right) shows iron-laden macrophages containing multiple ingested erythroblasts. The films provide a visual representation of the ineffective erythropoiesis of beta thalassemia major, showing the striking death of bone marrow erythroblasts. The macrophages are their graveyard.
Perioperative coagulopathy impacts patient outcome by influencing intraoperative hemodynamics and blood loss. We present two cases which despite having normal preoperative coagulation profiles and normotension during the surgery had intraoperative coagulopathy and unusual bleeding. These cases required a multidisciplinary approach to manage them successfully. A thorough knowledge of the coagulation cascade along with a high-degree of suspicion and early recognition of coagulation is required. Abnormalities are critical for a timely intervention. Point-of-care tests like thromboelastography (TEG) as well as platelet function studies helped us arrive at an early diagnosis and initiate prompt treatment.
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