A 38-year-old female presented to the emergency department of MKCG Medical College and hospital with chief complaints of a two day history of generalized headache associated with nausea, vomiting, blurring of vision and photophobia. This was associated with one episode of generalized tonic-clonic seizure a few hours prior to arrival at the ER. Her previous history was not suggestive of any prior episode of transient ischemic attacks, stroke or seizures. She was diagnosed as a case of Ulcerative Colitis (UC) two years back. She was on oral steroids, 5-Amino Salicylic Acid (5-ASA) and sulfasalazine following which she was symptom-free for last one year.On examination she was afebrile and normotensive. Glasgow Coma Scale (GCS) was 11/15 (E3V4M4) with bilateral plantar extensor. Fundoscopy revealed changes suggestive of early papilloedema in both eyes. Routine tests revealed a normocytic normochromic anaemia with a haemoglobin of 9.8 g%, total leukocyte count of 10,400/mm 3 , Erythrocyte Sedimentation Rate (ESR) of 18mm in 1 st hour, platelet count was 3.01 lakhs/mm 3 . C-Reactive Protein (CRP)was 10.6 mg/dl. Prothrombin Time (PT) was 13.0 seconds (Reference range: 12-13 seconds), International Normalised Ratio (INR) was 1.45, activated partial thromboplastin time (aPTT) was 36 seconds (Reference range: 32-40 seconds). Liver and renal function tests along with serum electrolytes were within normal limits. Sickling test was negative. Blood and stool cultures collected on the day of admission were negative for known pathogens. Stool routine and microscopy revealed few leukocytes and erythrocytes. Rectal examination was negative for melena, bleeding or diarrhoea. Non-Contrast Computerised Tomography (NCCT) scan of the head was normal. Owing to her young age and absence of known risk factors for stroke, cerebral venous sinus thrombosis was suspected and MRI venography was ordered which showed a filling defect in left transverse sinus and left sigmoid sinus [Table/ Fig-1]. A provisional diagnosis of Cerebral Venous Sinus Thrombosis (CVST) was made and she was immediately put on intravenous Low Molecular Weight Heparin (LMWH) therapy and phenytoin.Prothrombotic work-up and coagulation studies were negative for protein C and protein S activity, Antithrombin III activity, Anti-phospholipid antibody, Homocysteine and factor V Leiden

A 27-year-old, non-smoker male was admitted to the in-patient Department of Medicine of MKCG Medical College with complaints of intermittent fever not associated with chills and rigor for 8 days, shortness of breath {Medical Research Council (MRC) dyspnoea grade 2}, multiple purpuric spots over chest and extremities, epistaxis, bleeding per rectum, sub-conjunctival bleed and haematuria. There was no history of any haematological disorder, liver disease, cardiovascular disease or any medication intake in the recent past. Physical examinations revealed a temperature of 102°F, and respiratory rate of 28/minute. Blood pressure was 100/60mm Hg, no lymphadenopathy or hepatospleenomegaly. Chest wall examination revealed crepitation over right infra-clavicular area. Perrectal examination revealed no pathology. The electrocardiography was with-in normal limit. The initial complete blood count and routine biochemical parameters were normal [Table/ Fig-1,2]. Direct Coombs test was negative and Fibrin Degradation Product (FDP) was normal. Serum ferritin was 226ng/ml. (normal value 18-270ng/ml). Quantitative Buffy Coat and Immuno-cromatography Test for malarial antigens were negative. Antibodies to Salmonella antigen, Anti-neutrophlic antibody and rheumatoid factor were absent. HIV, Hepatitis B surface Antigen, Anti-Hepatitis C Virus antibody tests were negative. Blood culture was negative. Bone marrow biopsy showed hyper-cellular marrow and normal maturation of myeloid and erythroid precursors. Megakaryocytes were increased in number and had normal morphology. No granulomas, leukemic infiltrate or metastatic deposits were detected. Sputum was positive for Acid Fast Bacilli [AFB] on more than two occasions. A chest x-ray demonstrated patchy opacity over right upper zone [Table/ Fig-3]. Based upon above finding, a provisional diagnosis of pulmonary tuberculosis with thrombocytopenia was made. Patient was then put on Directly Observed Treatment and Short (DOTS) course category-1 Anti-Tuberculosis Therapy (ATT) (i.e., isoniazid 5mg/ kg/d, rifampicin10mg/kg/d, ethambutol 25mg/kg/d, pyrazinamide 30mg/kg/d) and subsequently received 8 units of random donor platelets. However, over a period of next 7 days the platelet counts did not increased satisfactorily and the petechiae and purpura were remained as before. In view of low platelet count oral prednisone (1mg/kg/day) was started along with ATT. By judging the clinical severity and patient's affordability for human immunoglobulin in a resource limited setting like our institution, we had preferred prednisolone as a primary therapy.Platelets count started to increase after two weeks of the ATT and prednisone. There was no fresh bleeding from any sites, petechiae and purpura started to decrease. Prednisone was then tapered off over a period of one month. Patient was followed-up every month for first 6 months and there was no recurrence of thrombocytopenia. Sputum AFB was negative at 2 nd and 6 th months of follow-up. Platelet counts were 1.54lacs/Cumm, 2.24lacs/Cumm at first and sec...

A 60-year-old male presented to the Department of Medicine of MKCG Medical College with complaints of sudden onset, severe localized pain over the distal part of fingers and toes of both the hands, feet and tip of the nose. The hands were affected more than the feet, right hand more than the left, 2 nd and 3 rd digits more than the rest of the fingers [Table/ Fig-1]. The pain gradually increased in intensity over the last two days, did not subside by analgesics and was associated with sensation of pins and needles over the affected parts. He also complained of joint pain over bilateral knee, ankle, distal interphalangeal and metacarpophalangeal joints of hand.There was no history of fever, intake of ergotamine drugs or beta blockers, any insect bite, or living at high altitude at any point of time. There was no history of diabetes, hypertension, peripheral vascular diseases, respiratory ailment, rheumatoid arthritis, Raynaud's phenomenon or any other connective tissue disorders. He was not a smoker.General physical examination was unremarkable. Pulse was 84/ minute, regular and all peripheral pulses were well felt. Blood pressure was 130/80 mm Hg. Systemic examination was also normal except for decreased touch, pain and temperature sensation over the tips of fingers and toes. The affected parts revealed a cold, cyanosed distal phalanx of both hands and feet. Allen's and Adson's tests were negative.A provisional diagnosis of symmetrical peripheral gangrene was made.Complete haemogram along with peripheral smear revealed dimorphic anemia [ ABSTRACTCryoglobulins are monoclonal or polyclonal immunoglobulins that undergo reversible precipitation at low temperatures. Cryoglobulinemia is associated with HCV infection in more than 90% cases, the remaining 10% being called as Essential Cryoglobulinemia which is generally associated with a severe course and suboptimal response to conventional therapies. As the digital vessels are more prone to colder temperatures, hyperviscosity in those vessels can initiate local thrombosis and may manifest as ischemic ulceration and gangrene. We report here a very unusual case of HCV negative cryoglobulinemic vasculitis presenting as symmetrical peripheral gangrene of fingers and toes.Keywords: Acronecrosis, Essential mixed cryoglobulinemia, Symmetrical digital gangrene, Vasculitis.normal. Serum antibodies against malarial parasite, HIV, Hepatitis B and Hepatitis C virus were absent. C-Reactive Protein (CRP) and Rheumatoid factor were positive.Antinuclear antibody (ANA) came out to be positive (47.2)(Positive = >23 IU/ml). Anti ds-DNA, anti Smooth muscle Ab, anti Scl-70 were negative. Complement studies revealed a normal C3 levels (110 mg/dL) (Reference range: 88-252 mg/dl) and low C4 levels (8 mg/dL) (Reference range: 12-72 mg/dl). Bone marrow aspiration study revealed hypercellular marrow with no evidence of metastatic deposits or leukemia. Reactive plasma cells were increased in number.Serum was then evaluated for the presence of cryoglobulins (qualitative) which revealed presence o...

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