Non-traumatic extraocular muscle haemorrhage associated with anticoagulation use.

We present a case of a unilateral extraocular muscle haematoma in an adult female patient who was compliant with life-long oral anticoagulation for recurrent deep vein thrombosis. The patient presented with symptoms of sudden-onset left-sided headache radiating to the temporal region, which started 2 days prior. No obvious triggering factors were identified. Cranial and ocular examinations were within normal limits. Imaging revealed a haemorrhage related to the lateral rectus muscle of the left eye. Conservative management was employed with abstinence from anticoagulation for 2 weeks and a weaning regime of oral steroids. Under the clinical review of ophthalmology and interval radiological monitoring, symptoms were reduced with reduction of haemorrhage size. Anticoagulation was reinstated after 2 weeks. To our knowledge, this is the first case of a non-traumatic extraocular muscle haematoma to be reported in a patient on anticoagulation.

Immune thrombocytopaenic purpura following the second dose of Pfizer COVID-19 vaccine

Immune thrombocytopaenic purpura (ITP) is often a diagnosis of exclusion with presentations ranging widely from asymptomatic patients to those with life-threatening bleeding. Secondary ITP following vaccination is relatively uncommon and underdiagnosed as majority of patients remain asymptomatic. Cases of severe thrombocytopaenia associated with SARS-CoV-2 messenger RNA (ribonucleic acid) vaccinations have been described previously, mostly as isolated occurrences, and typically occurring following the first dose. Here we present a case of severe ITP associated with the second dose of the Pfizer-BioNTech/BNT16B2b2 mRNA vaccine and provide a review of the current literature.

Severe case of refractory immune thrombocytopenic purpura requiring splenectomy after the COVID-19 vaccine.

Immune thrombocytopenic purpura (ITP) is an autoimmune disorder caused by autoantibodies against platelet antigens resulting in platelet destruction and inhibition of platelet production. Occasionally, an inciting event such as a virus or vaccination can precipitate ITP. Several cases of ITP have been reported after the BTN162b2 (Pfizer-BioNTech) and mRNA-1273 (Moderna) SARS-CoV-2 (COVID-19) vaccines. All reported cases of post-vaccination ITP have resolved with medical therapy until this case.A man in his mid-20s developed bleeding from ITP 2 weeks after receiving the second dose of the Pfizer SARS-CoV-2 vaccine. All inpatient medical treatment attempts failed. On hospital day 40, a splenectomy was performed and he ultimately recovered and was discharged.Awareness of potential vaccination side effects is a fundamental element of refining and improving patient safety. This case illustrates that given the right circumstances, serious refractory ITP can develop in response to the second dose of the Pfizer SARS-CoV-2 vaccine.

Cold agglutinin disease secondary to severe SARS-CoV-2 treated with eculizumab.

Impaired immune response with uncontrolled inflammation and various immunological disorders have been reported during SARS-CoV-2 infection. Here, we report a case of cold agglutinin disease occurring during a severe coronavirus disease 2019 (COVID-19) in a French intensive care unit. A patient was presented with acute respiratory distress syndrome, acute renal failure and haemolytic anaemia. Direct antiglobulin test was positive with a cold agglutinin titre of 1/512. No other cause than COVID-19 explained the occurrence of cold agglutinin disease; however, causality could not be formally established. Persistent anaemia despite transfusion therapy and the short-term life-threatening, prompted the infusion of a monoclonal anti-C5 antibody (eculizumab). Eculizumab therapy quasi-fully resolved haemolysis within a few days, but ultimately the patient died from his severe COVID-19 infection. Data regarding the specific treatment of cold agglutinin disease during COVID-19 are rare. Although additional studies are warranted, eculizumab may be considered in critical situations.

Vaccine-induced thrombosis and thrombocytopaenia with widespread abdominal venous thrombosis, venous ischaemia and bowel oedema.

A 49-year-old woman presented with severe abdominal pain and per rectal bleed, 13 days after receiving the first dose of the AstraZeneca vaccine. Blood tests showed remarkably low platelet count, unmeasurable D-dimer levels and low fibrinogen levels, consistent with a diagnosis of vaccine-induced thrombotic thrombocytopaenia and disseminated intravascular coagulation. CT mesenteric angiogram revealed massive portosplenic mesenteric vein thrombosis. CT head also noted non-occlusive thrombosis at several sites. She was treated with intravenous immunoglobulins, plasma exchange, anticoagulants and transjugular intrahepatic portosystemic shunt procedure. Following a prolonged inpatient stay, she was discharged with subsequent short bowel syndrome and long-term parenteral nutrition. This particular clinical scenario aims to highlight the importance for clinicians to remain vigilant for rare complications associated with the AstraZeneca vaccine and the subsequent management involved, at a time where it is vital to vaccinate globally in order to control the spread of the COVID-19 pandemic.

Haemophagocytic lymphohistiocytosis following COVID-19 mRNA vaccination.

The development of vaccinations has been instrumental in the ongoing effort to combat the COVID-19 pandemic. Although the benefits of vaccination are unquestionable, there have been reports of potentially rare life-threatening complications following vaccination including thrombocytopaenia, haemolytic anaemia, vasculitis and myocarditis. Haemophagocytic lymphohistiocytosis (HLH), a rare but life-threatening inflammatory condition, has also been described postadenoviral vector COVID-19 vaccination but it has never been reported post-messenger RNA (mRNA) COVID-19 vaccination. We report two cases of HLH admitted to our hospital after administration of COVID-19 mRNA vaccines. We also searched the vaccine adverse event reporting system and found 50 reports of suspected HLH following COVID-19 vaccination. Presently, we cannot define a causality between COVID-19 mRNA vaccination and HLH development. However, we hope the reporting of our two cases (and additional cases seen in the adverse event reporting database) will help us determine whether there is a potential relationship. Prompt recognition of this condition is of utmost importance to initiate life-saving therapy.

Lamotrigine-associated haemophagocytic lymphohistiocytosis (HLH) confounded with underlying rheumatoid arthritis.

Haemophagocytic lymphohistiocytosis (HLH) is one of the rare haematological syndromes more commonly reported in infants/children than adults. This disease is known for its aggressive dysregulated immune response affecting the host rapidly, causing multiorgan dysfunction and thus carries a high mortality. The disease still remains cryptic in this current decade despite all the developments in the ever-evolving field of haematology. Due to its rare occurrence and being more frequent in infants and the paediatric population, the literature lacks enough data to standardise therapies. Such events in adults and the elderly are invariably related to an underlying insult such as infections, other autoimmune or rheumatological diseases or drugs. We describe an interesting case of a middle-aged Caucasian woman who presented with fever, pancytopenia and hepatitis, who was eventually diagnosed with HLH just in time to receive the life-saving specific treatment as per available guidelines.

Thrombotic thrombocytopenic purpura after ChAdOx1 nCoV-19 vaccine.

A 50-year-old Indian woman presented with acute dysphasia, left upper limb numbness and thrombocytopenia 12 days after receiving the ChAdOx1 nCoV-19 vaccine (AstraZeneca/Vaxzevria). MRI of the brain was unremarkable. Microangiopathic haemolytic anaemia with thrombocytopenia was noted on her peripheral blood film. A diagnosis of thrombotic thrombocytopenic purpura (TTP) was confirmed through the findings of absent ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity and markedly raised titre of ADAMTS13 autoantibodies. Prompt treatment with plasma exchange, adjunctive steroids and rituximab was commenced. A remission of TTP was achieved and she was discharged 3 weeks after admission. While other immune-mediated conditions have been documented after receipt of the vaccine, this report highlights the first case of immune-mediated TTP diagnosed after administration of the ChAdOx1 nCoV-19 vaccine.

Rare case of COVID-19 vaccine-associated intracranial haemorrhage with venous sinus thrombosis.

Vaccine-induced immune thrombotic thrombocytopenia (VITT) is a relatively novel term which describes patients who have developed a low platelet count and prothrombotic tendencies secondary to receiving a vaccine. The concept has been derived from the well-established phenomenon of heparin-induced thrombocytopenia, and several cases of VITT have now been reported in patients who have received the AstraZeneca (ChAdOx1 nCov-19) vaccine. Unfortunately, some of these patients have gone on to develop intracranial venous sinus thrombosis. We present a case of VITT-associated sinus thrombosis secondary to the AstraZeneca (ChAdOx1 nCov-19) vaccine, which was complicated by a large intracerebral haemorrhage.

Fungal central skull-base osteomyelitis: atypical presentation and management issues.

Mucormycosis is a very commonly encountered disease in the sinonasal region in patients with diabetes and immunocompromised status specially in the northern part of the Indian continent. Due to its fulminant nature and involvement in the rhino-orbital-cerebral region, prognosis is poor even after extensive debridement and amphotericin-B therapy. We present a case with diagnosis of sarcoidosis being treated with systemic steroids who developed sudden-onset ptosis and left lateral rectus palsy. On radiological evaluation with MRI, a heterogenously peripherally enhancing lesion was seen in the nasopharynx suggestive of nasopharyngeal abscess. The patient was taken up for emergency surgery; a necrotic lesion with destruction of sphenoid bone, pterygoids and clivus was seen originating in the nasopharynx. The lesion on fungal staining came out to be broad aseptate hyphae suggestive of mucormycosis.

Severe acquired haemophilia associated with asymptomatic SARS-CoV-2 infection.

A 65-year-old man presented with symptoms of severe subcutaneous bleeding in his arm, which led to compartment syndrome requiring fasciotomy and massive blood transfusion protocol. Medical history was significant for history of autoimmune thyroid disease. Workup revealed elevated partial thromboplastin time, decreased factor VIII levels and elevated factor VIII inhibitor levels. He was worked up for causes of acquired haemophilia A and was found to have an elevated SARS-CoV-2 antibody level. Given his negative workup for other secondary aetiologies, we suspect that the cause of his haemophilia A was from his SARS-CoV-2 infection, which has been observed previously in various case reports.

COVID-19-associated ischaemic stroke due to underlying carotid artery thrombosis in a healthy young woman.

SARS-CoV-2 has proven its versatility in host presentations; one such presentation is a hypercoagulable state causing large-vessel thrombosis. We report a case on a previously asymptomatic COVID-19-positive patient presenting with an acute ischaemic stroke and an incidental left internal carotid artery thrombus. The patient's medical, social and family history and hypercoagulability screening excluded any other explanation for the left carotid thrombus or stroke, except for testing positive for the COVID-19. This case explores the known hypercoagulable state associated with COVID-19 and the effect of the virus on the host's immune response. It also questions whether administration of recombinant tissue plasminogen activator (t-PA), according to the American Heart Association guidelines, following a negative head CT for haemorrhagic stroke is safe without prior extended imaging in this patient population. We recommend, in addition to obtaining a non-contrast CT scan of the brain, a CT angiogram or carotid duplex of the neck be obtained routinely in patients with COVID-19 exhibiting stroke symptoms before t-PA administration as the effects may be detrimental. This recommendation will likely prevent fragmentation and embolisation of an undetected carotid thrombus.

Cold agglutinin disease and COVID-19 requiring therapeutic plasma exchange.

A 70-year-old man with known cold autoimmune haemolytic anaemia was referred to the emergency department with increased shortness of breath on exertion. He had been confirmed positive for non-variant COVID-19 infection 1 week earlier based on nasopharyngeal swab PCR assay. CT thorax demonstrated diffuse patchy bilateral ground glass opacities, consistent with COVID-19 pneumonia. Bloodwork demonstrated severe cold agglutinin mediated haemolytic anaemia. To help stabilise the patient, he was transferred to a tertiary care hospital for urgent therapeutic plasma exchange. Key supportive therapy included folic acid supplementation, ensuring the patient was kept warm and warmed infusions including transfusions via the apheresis machine. The patient made a good recovery following plasma exchange, and his haemoglobin levels remained stable by discharge.

Rapid onset vancomycin-induced thrombocytopenia confirmed by vancomycin antibody test.

We report a case of vancomycin-induced thrombocytopenia (VIT) with rapid onset after re-exposure to vancomycin. A 58-year-old man with cellulitis was initiated on vancomycin. Approximately 1 hour into the vancomycin infusion, the patient developed an infusion-related reaction. Vancomycin infusion was stopped. A complete blood count obtained 4 hours after discontinuation of the vancomycin infusion revealed a platelet count of 31 ×10-9/L. Investigations ruled out likely causes of thrombocytopenia. VIT was diagnosed based on clinical symptoms and confirmed with drug-dependent platelet antibody testing. Without complications, platelet counts recovered within 7 days after discontinuation of vancomycin. No correlation between vancomycin level and VIT was observed.

Autoimmune heparin-induced thrombocytopenia: a rare manifestation of COVID-19.

We describe the case of a 65-year-old male who presented to an outside hospital for shortness of breath, nausea and vomiting 8 days after testing positive for COVID-19. Initial workup revealed massive bilateral pulmonary emboli and thrombocytopenia. The patient was then admitted to our hospital, received an inferior vena cava filter and initially started on argatroban for autoimmune heparin-induced thrombocytopenia (HIT) prophylaxis. On hospital stay day 6, labs revealed a diagnosis of HIT in the setting of COVID-19. This case highlights the rare occurrence of a patient developing HIT without heparin exposure and in the setting of a novel infectious agent, COVID-19.

Delayed, transient and self-resolving neutropenia following COVID-19 pneumonia.

A novel coronaravirus, identified as SARS-CoV-2, spread throughout the world in 2020. The COVID-19 pandemic has led to many discoveries and clinical manifestations. A young patient is presented with new, self-resolving neutropenia presenting weeks after a prolonged hospital stay for COVID-19 pneumonia. Workup included analysis for underlying infection, nutritional abnormalities, malignancy, medication and toxin exposure, all of which were negative. From 2020 to the present, few reports have described neutropenia associated with a recent COVID-19 infection. In particular, no reports have described a delayed presentation of neutropenia. The authors would like to propose that the significant inflammatory response associated with COVID-19 is likely what led to this patient's postviral neutropenia. Furthermore, in young healthy patients, bone marrow biopsy may be deferred and a watchful-waiting approach may be taken to assess for neutropenia resolution.

Febrile neutropenia due to COVID-19 in an immunocompetent patient.

While lymphopenia has been a common finding in COVID-19 infection, particularly in severe cases, febrile neutropenia has been very rarely reported in immunocompetent patients with COVID-19. Herein, we report the case of a 76-year-old hypertensive and diabetic man who was hospitalised with severe COVID-19 infection and developed delayed-onset severe neutropenia with neutropenic fever, which responded to treatment with antibiotics and granulocyte colony-stimulating factor. This case highlights the importance of identifying a rare complication (febrile neutropenia on the fifth week) of COVID-19 infection in hospitalised patients by intensive monitoring and aggressive management for favourable outcomes.

Rare case of pancrelipase therapy-induced neutropaenia.

A 61-year-old man was transferred to our facility from an outside hospital due to refractory neutropaenia of unknown aetiology. The patient presented to the referring hospital with a 5-day history of worsening diarrhoea and abdominal pain. Initial lab results at presentation showed severe neutropaenia with an absolute neutrophil count of 0. Investigations included a bone marrow biopsy which showed slightly hypocellular marrow with near absence of granulocytic precursors. A CT without contrast showed evidence of chronic pancreatitis and acute colitis. The patient's neutropaenia persisted despite granulocyte colony-stimulating factor therapy. The patient was, thus, transferred to our facility for a higher level of care. At our facility, the patient had rapid correction of neutropaenia after discontinuation of pancrelipase therapy. The patient's abdominal pain and diarrhoea also improved while off pancrelipase. Neutropaenia has completely resolved 6 weeks after discharge without any further therapy.

Cardiac amyloidosis masquerading as acute coronary syndrome.

A 53-year-old man presented to a district general hospital with chest pain, ECG changes and a small high-sensitivity cardiac troponin I rise. There were no symptoms of heart failure. CT coronary angiography revealed moderate calcific disease and conventional angiography confirmed no flow limitation. Echocardiography showed left ventricular hypertrophy (LVH). His blood pressure remained normal throughout his admission. The tertiary centre labelled this as a 'plaque rupture' event but the LVH remained unexplained. Cardiac MRI displayed an unusual pattern of late gadolinium enhancement, which was not classical of amyloid. However, a raised serum free kappa light chain along with the deposition of amyloid on his bone marrow aspirate confirmed the diagnosis of primary AL amyloidosis with cardiac involvement. The patient went on to have chemotherapy and remained stable at 1-year follow-up.

Acute kidney injury and nephrotic syndrome associated with eltrombopag therapy in chronic idiopathic thrombocytopenic purpura.

A 77-year-old man was admitted with severe acute kidney injury and nephrotic syndrome. He was started on eltrombopag for chronic idiopathic thrombocytopenic purpura 6 weeks earlier. An ultrasound of the kidneys was normal and an auto-antibody screen was negative. The use of the Naranjo adverse drug reaction probability scale indicated a probable relationship (score of 5) between the patient's development of acute renal failure and eltrombopag therapy. Literature review identified only one other case of nephrotic syndrome and acute kidney injury associated with eltrombopag therapy in which a kidney biopsy revealed focal segmental glomerulosclerosis. Due to the challenges faced during the prevailing SARS-CoV-2 pandemic and persistent low platelet counts a renal biopsy was not undertaken. On stopping eltrombopag, the patients renal function stabilised and he successfully went into remission following treatment with high dose corticosteroids and diuretics. This report of a serious case of reversible renal failure and nephrotic syndrome after treatment with eltrombopag may serve to inform clinicians about the possible severe renal adverse effects of eltrombopag before its commencement for future use.