Rituximab induced acute thrombocytopenia in a patient with systemic lupus erythematosus: a case report.

BACKGROUND: Rituximab is a novel chimeric monoclonal antibody that has established itself as a potent therapeutic option for autoimmune medical conditions, including systemic lupus erythematosus, owing to its mechanism of action targeting CD20 cells. Rituximab is also known to cause a spectrum of side effects including hematological abnormalities. Acute isolated thrombocytopenia following rituximab is an uncommon occurrence and, when seen, occurs in the presence of underlying hematological malignancies. Its occurrence in autoimmune diseases is rare. Despite this, acute isolated thrombocytopenia in the backdrop of systemic lupus erythematosus is undocumented. CASE PRESENTATION: A young 36-year-old South Asian female with systemic lupus erythematosus with class IV lupus nephritis poorly responding to standard therapy was initiated on rituximab. Ten days later, she presented with mucocutaneous bleeding and ecchymotic skin lesions. Isolated severe thrombocytopenia was noted with a platelet count of 5 × 109/L (150-450). Anticipating life-threatening bleeding, she was given intravenous immunoglobulin, methyl prednisolone, and platelet transfusion considering a spectrum of initial differential diagnosis. Rituximab was also withheld. Though extensively investigated, most investigations were negative. A platelet destructive process was suspected as bone marrow biopsy showed adequate megakaryocytes. Weighing the risk versus benefit, following recovery, she was reinitiated on rituximab. Within 4 days, she presented again with similar symptoms and severe isolated thrombocytopenia was noted. Rituximab-induced acute thrombocytopenia was considered the working clinical diagnosis. CASE DISCUSSION AND CONCLUSION: Rituximab can cause a spectrum of hematological abnormalities, including isolated acute thrombocytopenia. Its occurrence in autoimmune conditions is rare, and its manifestation in systemic lupus erythematosus is undocumented. Its exact etiology is still disputed. Usually considered benign, the platelet numbers tend to show improvement with cessation of therapy. However, in the presence of mucocutaneous bleeding in our patient, we took an aggressive approach to management. Though evidence for corrective therapy is anecdotal, it could be justified on the basis of averting potential catastrophic hemorrhagic manifestations. The spectrum of autoimmune disease that potentially predisposes rituximab to cause thrombocytopenia should be extended to include systemic lupus erythematosus.

The eye in dengue fever, a rarely appreciated aspect of dengue expanded syndrome: a case report.

BACKGROUND: Dengue fever is a mosquito-borne illness prevalent mainly in the tropics. It is feared for causing the dengue hemorrhagic spectrum of the disease leading to significant morbidity and mortality. Its rarer manifestations are categorized as the expanded dengue syndrome, and though being recognized, they are not fully appreciated and understood. The involvement of the eye in dengue fever is one such phenomenon. CASE PRESENTATION: A 27-year-old South-Asian woman presented on day 2 of dengue fever, without capillary leakage, for further management. Despite developing hepatitis, she had an otherwise uncomplicated progression of the illness because she did not develop capillary leakage. On day 8 of the illness, she had the lowest platelet count and developed bilateral blurred vision. Examination revealed that only gross movements were detected in the left eye, and the right eye had a visual acuity of 6/9. She was diagnosed with foveolitis in the right eye and central serous chorioretinopathy in the left eye, along with hemorrhages in both eyes. These were confirmed by funduscopy, fluorescein angiography, optical coherence tomography, and macular scans. She received systemic and intravitreal steroids and was assessed regularly. After 6 months of observation, her visual acuity was 6/6 in the right eye and 6/9 in the left eye, which remained the same thereafter. DISCUSSION: The exact mechanism of eye involvement in dengue viral infection is poorly understood. Multiple causes have been suspected and include viral factors, immune mediation, capillary leakage, stress, and hemorrhage. Eye involvement is classically seen at the lowest platelet count and when the count begins to rise. Though symptoms are nonpathognomonic, blurring of vision is the commonest complaint, but the range of presentation is extensive and variable. Ophthalmological assessment and funduscopy are very useful in addition to advanced assessments. There is no clear consensus on management; suggestions range from conservative care to aggressive steroid therapy with immune modulation and even ophthalmological intervention. Recovery can be full or partial with a variable time scale. CONCLUSION: The extensive spectrum of possible visual symptoms should prompt the clinician to suspect any visual complaint as potential dengue eye involvement. Guided studies and screening are needed to better understand the true incidence of eye involvement in dengue fever.

Lucio's phenomenon, an uncommon occurrence among leprosy patients in Sri Lanka.

BACKGROUND: Lucio's phenomenon is a rare manifestation of untreated leprosy which is seen almost exclusively in regions surrounding the Gulf of Mexico. Its occurrence elsewhere though documented is considered uncommon. We present a case of Lucio's phenomenon in a previously undiagnosed leprosy patient who presented to us with its classical skin manifestations. CASE PRESENTATION: A 64 year old South Asian (Sri Lankan) male with a history of chronic obstructive airway disease presented to us with fever and cough. He had a generalized smooth and shiny skin with ulcerating skin lesions afflicting the digits of the fingers. The lesions progressed to involve the extremities of the body and healed with crusting. Based on the clinical and investigational findings Tuberculosis and common vasculitic conditions were suspected and excluded. The unusual skin manifestations prompted a biopsy, and wade fite stained revealed Mycobacterium bacilli. In context of the clinical picture and histological findings, Lucio's phenomenon was suspected. A clinical diagnosis of Lucio's phenomenon occurring in the backdrop of lepromatous leprosy was made. CONCLUSION: Though leprosy is still a prevalent disease, it has manifestations that are not easily recognized or fully appreciated. Regional patterns of atypical manifestations should not limit better understanding of rarer manifestations as it will aid in clinching an early diagnosis and instituting prompt treatment, thereby reducing morbidity and mortality.

A case of methyl ethyl ketone peroxide poisoning and a review of complications and their management.

Methyl Ethyl Ketone Peroxide (MEKP) is a highly toxic clear liquid used as a solvent. It is a strong oxidizing agent and a corrosive. Acute and chronic toxicity can occur as an occupational hazard. Ingestion is associated with corrosive burns leading to stricture formation, inhalational pneumonitis, acidosis, liver failure and renal failure. In this paper we present a case of a young patient who intentionally ingested MEKP. The patient developed multiple complications including proximal intestinal obstruction, acidosis and acute kidney injury. He was managed conservatively and recovered after a prolonged hospital stay. He had multiple inflammatory strictures on esophageal endoscopy, which improved over 3-6 moths.

Complete heart block in dengue complicating management of shock due to both bleeding and leakage: a case report.

BACKGROUND: The spread of Dengue virus infection is reaching pandemic proportions. Dengue is usually dreaded for causing shock due to capillary leakage. However the clinical spectrum of dengue is vast and the newly incorporated expanded dengue syndrome introduces a wide range of presentations that are rarely observed and appreciated but nevertheless have the potential to cause significant morbidity and even mortality. Cardiac involvement in dengue is one such example. CASE PRESENTATION: A 26 year old South-Asian female presented in a state of haemodynamic shock with a history of fever and use of non-steroidal anti inflammatory drugs. Dengue was suspected clinically and later confirmed. Following stabilization and while still in the febrile phase the patient developed bradycardia with dynamic electrocardiogram changes which evolved into complete heart block. However there was no circulatory compromise. Clinical picture was further complicated by the development of dengue haemorhaghic fever and cautious fluid resuscitation was carried out in correlation to clinical and haematological parameters. Impaired coagulation profile necessitated administration of activated factor seven on the backdrop of low platelets and bleeding. Cardiac pacing could be avoided due to maintenance of vitals within acceptable parameters. CONCLUSION: Expanded dengue syndrome should be given greater appreciation as not all may be benign. Cardiovascular system involvement in dengue has the potential to cause significant morbidity and mortality. Careful interpretation of clinical parameters will help in the institution of the appropriate management and help avoid unnecessary invasive interventions. Screening of dengue patients with timely electrocardiographs would be useful to detect cardiac involvement. Guidance on managing atypical manifestations of dengue expanded syndrome should available to help clinicians dictate treatment.

Portal vein thrombosis associated with psoriasis: a case report.

BACKGROUND: Psoriasis is no longer viewed as an isolated dermatological ailment and instead is considered a systemic disease. The extension of this spectrum has heightened the known risk of morbidity and mortality due to the involvement of cardiovascular system and the risk of venous thrombosis. A number of cases have reported the increased occurrence of deep vein thrombosis and pulmonary embolism in the background of psoriasis, however portal vein thrombosis has not been reported to date. We report an index case of chronic portal vein thrombosis in a diagnosed patient with psoriasis. CASE PRESENTATION: A 67-year-old South-Asian female previously diagnosed and treated for psoriasis presented with a four month history of abdominal pain associated with abdominal distension. Clinical examination revealed an enlarged spleen and free fluid in the abdomen. Imaging with ultrasonography and computed tomography of the abdomen revealed features compatible with chronic portal vein thrombosis with cavernous transformation. CONCLUSION: This case highlights the importance of having clinical awareness of occurrence of thrombosis in patients with psoriasis. Typical symptoms favoring thrombosis should prompt thorough investigation to exclude this rare yet possible complication in patients with psoriasis, including that of portal vein thrombosis. Prophylaxis with anticoagulation still lacks strength of evidence to be justified in psoriasis. The exact pathogenesis of venous thromboembolism in psoriasis is still unexplained and further studies are needed to clarify the causal association.

Inoperable inflammatory myofibroblastic tumour of the para-nasal sinuses and orbit with recurrence responding to methotrexate and prednisolone: a case report.

BACKGROUND: Inflammatory myofibroblastic tumour is a rare neoplasm with a potential to behave in a malignant manner. It can occur anywhere in the body, however involvement of the head, especially the para-nasal sinuses is rare. CASE PRESENTATION: A 33-year-old South Asian male presented with coryzal symptoms including a persistent cough with an asymmetrical swelling of the left side of the face. Imaging revealed a mass lesion involving the para-nasal sinuses eroding into the orbit. Histology and the clinical picture were compatible with inflammatory myofibroblastic tumour. As curative excision of the tumour was not feasible, medical management was offered. Despite early features of remission to glucocorticoids, tapering resulted in recurrence. Hence combination therapy with glucocorticoids and methotrexate was commenced with dramatic reduction of tumour burden and the patient has been in remission to date. CONCLUSION: Inflammatory myofibroblastic tumour has the potential to behave in a malignant manner. Medical management with chemotherapy, glucocorticoids and non-steroidal anti-inflammatory drugs though effective, do not have a uniform response pattern. Surgically unresectable inflammatory myofibroblastic tumour above neck should be treated aggressively with combination regimens. Combination of prednisolone with methotrexate has been shown to have good outcome.

Dengue haemorrhagic fever presenting with cholestatic hepatitis: two case reports and a review of literature.

BACKGROUND: Dengue fever is a common mosquito borne viral fever in South Asia, which causes significant morbidity and mortality. Dengue fever is well known to involve the liver, especially in dengue hemorrhagic fever. The hepatic involvement is usually that of a mild hepatitis with transaminase derangement without jaundice. In cases of dengue hemorrhagic fever where shock has ensued, a severe hepatitis with gross derangements of transaminases and bilirubin may occur. These are two rare cases of adult patients with dengue hemorrhagic fever presenting with a cholestatic type of jaundice. CASE PRESENTATION: This case report describes two female patients aged 30 and 46 years who presented with fever, icterus and biochemical analysis revealed cholestatic jaundice. Evolution of the clinical picture and dropping platelets prompted serological investigations in the form of dengue non-structural protein 1 antigen and dengue immunoglobulin M which confirmed acute dengue infection. CONCLUSION: These cases highlight the importance of considering dengue fever as a differential diagnosis even in the presence of a cholestatic jaundice, especially in countries where dengue fever is endemic, and in travelers returning from dengue endemic countries. The early diagnosis of dengue fever and timely institution of supportive fluid management is essential to prevent morbidity and mortality.

Sensory neuronopathy complicating systemic lupus erythematosus: a case report.

INTRODUCTION: Systemic lupus erythematosus is a multi-system connective tissue disorder. Peripheral neuropathy is a known and underestimated complication in systemic lupus erythematosus. Ganglionopathy manifests when neuronal cell bodies in the dorsal root ganglion are involved. Autoimmune disorders are a known etiology, with systemic lupus erythematosus being a rare cause. CASE PRESENTATION: A 32-year-old South Asian woman presented with oral ulceration involving her lips following initiation of treatment for a febrile illness associated with dysuria. She had a history of progressively worsening numbness over a period of 4 months involving both the upper and lower limbs symmetrically while sparing the trunk. Her vibration sense was impaired, and her reflexes were diminished. For the past 4 years, she had had a bilateral, symmetrical, non-deforming arthritis involving the upper and lower limbs. Her anti-nuclear antibody and anti-double-stranded deoxyribonucleic acid status were positive. Although her anti-Ro antibodies were positive, she did not have clinical features suggestive of Sjögren syndrome. Nerve conduction studies revealed sensory neuronopathy. A diagnosis of systemic lupus erythematosus complicated by sensory neuronopathy was made. Treatment with intravenous immunoglobulin resulted in clinical and electrophysiological improvement. CONCLUSION: Peripheral neuropathy in systemic lupus erythematosus can, by itself, be a disabling feature. Nerve conduction studies should be considered when relevant. Neuropathy in systemic lupus erythematosus should be given greater recognition, and rarer forms of presentation should be entertained in the differential diagnosis when the clinical picture is atypical. Intravenous immunoglobulin may have role in treatment of sensory neuronopathy in systemic lupus erythematosus.

Necrotizing fasciitis--a diagnostic dilemma: two case reports.

INTRODUCTION: Necrotizing soft tissue infections can affect various tissue planes. Although predisposing etiologies are many, they mostly center on impaired immunity occurring directly or indirectly and loss of integrity of protective barriers which predispose to infection. The nonspecific presentation may delay diagnosis and favor high mortality. CASE PRESENTATION: Two case vignettes are presented. The first patient, a 44-year-old healthy South Asian man with a history of repeated minor traumatic injury presented to a primary health care center with a swollen left lower limb. He was treated with antibiotics with an initial diagnosis of cellulitis. Because he deteriorated rapidly and additionally developed intestinal obstruction, he was transferred to our hospital which is a tertiary health care center for further evaluation and management. Prompt clinical diagnosis of necrotizing soft tissue infection was made and confirmed on magnetic resonance imaging as necrotizing fasciitis. Urgent debridement was done, but the already spread infection resulted in rapid clinical deterioration with resultant mortality. The second patient was a 35-year-old South Asian woman with systemic lupus erythematous receiving immunosuppressive therapy who developed left lower limb pain and fever. Medical attention was sought late as she came to the hospital after 4 days. Her condition deteriorated rapidly as she developed septic shock and died within 2 days. CONCLUSIONS: Necrotizing fasciitis can be fatal when not recognized and without early intervention. Clinicians and surgeons alike should have a greater level of suspicion and appreciation for this uncommon yet lethal infection.

Hump-nosed viper bite: an important but under-recognized cause of systemic envenoming.

Hump-nosed viper bites are common in the Indian subcontinent. In the past, hump-nosed vipers (Hypnale species) were considered moderately venomous snakes whose bites result mainly in local envenoming. However, a variety of severe local effects, hemostatic dysfunction, microangiopathic hemolysis, kidney injury and death have been reported following envenoming by Hypnale species. We systematically reviewed the medical literature on the epidemiology, toxin profile, diagnosis, and clinical, laboratory and postmortem features of hump-nosed viper envenoming, and highlight the need for development of an effective antivenom.

Hump-nosed viper bite: an important but under-recognized cause of systemic envenoming

Hump-nosed viper bites are common in the Indian subcontinent. In the past, hump-nosed vipers (Hypnale species) were considered moderately venomous snakes whose bites result mainly in local envenoming. However, a variety of severe local effects, hemostatic dysfunction, microangiopathic hemolysis, kidney injury and death have been reported following envenoming byHypnale species. We systematically reviewed the medical literature on the epidemiology, toxin profile, diagnosis, and clinical, laboratory and postmortem features of hump-nosed viper envenoming, and highlight the need for development of an effective antivenom.

Hump-nosed viper bite: an important but under-recognized cause of systemic envenoming

Hump-nosed viper bites are common in the Indian subcontinent. In the past, hump-nosed vipers (Hypnale species) were considered moderately venomous snakes whose bites result mainly in local envenoming. However, a variety of severe local effects, hemostatic dysfunction, microangiopathic hemolysis, kidney injury and death have been reported following envenoming byHypnale species. We systematically reviewed the medical literature on the epidemiology, toxin profile, diagnosis, and clinical, laboratory and postmortem features of hump-nosed viper envenoming, and highlight the need for development of an effective antivenom.