De novo pancytopaenia in an older adult with severe COVID-19 infection.

During the COVID-19 pandemic, it was recognised that SARS-CoV-2 can cause multisystem illness. Non-respiratory complications observed early in the pandemic were haematological in nature. A rare but serious haematological complication of COVID-19 infection is pancytopaenia. We describe a case of an older adult without pre-existing haematological disease or risk factors for cell dyscrasia with severe pancytopaenia induced by COVID-19, who developed critical illness requiring respiratory support in intensive care and died. Our case report highlights that de novo pancytopaenia may only present with mild dermatological manifestations and may indicate severe COVID-19 infection. Management is primarily supportive and early involvement of haematology should be sought.

HHV-8-associated multicentric Castleman disease with concurrent Kaposi sarcoma

Multicentric Castleman disease (MCD) is a rare lymphoproliferative disorder typically manifesting with bulky lymphadenopathy in multiple lymph node stations. We describe an atypical presentation of human herpes virus 8 (HHV8)-associated MCD in a middle-aged man with no significant medical history who presented with 1 month of systemic symptoms. He was found to be HIV-1 positive. A physical examination did not reveal palpable lymphadenopathy. A contrast-enhanced CT scan was notable for hepatosplenomegaly and mildly enlarged scattered lymph nodes in the abdomen and pelvis. A positron emission tomography/CT scan demonstrated hypermetabolic cervical chain lymph nodes. Posterior cervical lymph node pathology showed HHV8-positive MCD with concurrent HIV-associated Kaposi sarcoma. The patient was treated with rituximab and liposomal doxorubicin without response. We emphasise the lack of the hallmark of bulky lymphadenopathy in this patient, and the importance of a timely pathological diagnosis in MCD.

Severe refractory warm autoimmune haemolytic anaemia after the SARS-CoV-2 Pfizer-BioNTech vaccine (BNT162b2 mRNA) managed with emergency splenectomy and complement inhibition with eculizumab.

A male in his teens with a history of liver transplant for biliary atresia (aged 2 years) and autoimmune haemolytic anaemia (AIHA, aged 6 years) presented with jaundice, dark urine, fatigue and chest discomfort that began 48 hours after the first dose of SARS-CoV-2 Pfizer-BioNTech vaccine (BNT162b2 mRNA). Investigations revealed a warm AIHA picture. Over 4 weeks the patient developed life-threatening anaemia culminating in haemoglobin of 35 g/L (after transfusion), lactate dehydrogenase of 1293 units/L and bilirubin of 228 µmol/L, refractory to standard treatment with corticosteroids and rituximab. An emergency splenectomy was performed that slowed haemolysis but did not completely ameliorate it. Eculizumab, a terminal complement pathway inhibitor, was initiated to arrest intravascular haemolysis and showed a favourable response. AIHA is rare but described after the SARS-CoV-2 Pfizer-BioNTech vaccine. This case highlights the rare complication of AIHA, the use of emergency splenectomy for disease control, and the use of eculizumab.

Monoclonal gammopathy of undetermined significance causing large vessel vasculitis.

A man in his late 50s presented with unilateral pain and discolouration of his fourth and fifth toes suggestive of digital ischaemia. He had a medical history of two unprovoked venous thromboembolisms in the preceding 18 months and a history of monoclonal gammopathy of undetermined significance (MGUS). A CT scan showed evidence of large vessels vasculitis in the absence of circulating antineutrophil cytoplasmic antibodies. Biopsy of the toes showed evidence of light chain and immunoglobulin deposition on immunofluorescence suggesting vasculitis secondary to his haematological diagnosis of MGUS. The patient was treated with high dose glucocorticoids and immunosuppressive treatment with a significant improvement in his symptoms and features of digital ischaemia.

Superwarfarin poisoning: challenges still remain.

Superwarfarin (long-acting anticoagulant rodenticide) poisoning should be suspected in unexplained bleeding with prolonged prothrombin time, especially in the absence of another explanation. Diagnosis and treatment of this intoxication remain a challenge as the direct analysis of superwarfarin in serum is not always possible. Therefore, toxin bioavailability remains unknown and close monitoring and treatment for long periods are required to avoid serious bleeding complications. Here, we discuss a case of suspected superwarfarin poisoning to highlight the challenges in early diagnosis and the challenges we encountered in treatment management and ensuring compliance for long periods.

Management of a severe bilateral pulmonary embolism as a complication of VITT following vaccination with AstraZeneca COVID-19 vaccine.

Since the start of vaccination against COVID-19 viral infection using adenovirus-based vector vaccine (eg, The Oxford-AstraZeneca vaccine, using the modified chimpanzee adenovirus ChAdOx1, and the Johnson & Johnson vaccine, using human adenovirus serotype 26), a rare, but potentially life-threatening complication called vaccine-induced thrombotic thrombocytopenia (VITT) was reported. As the number of cases increases every day, with the increase in the number of vaccinated people all over the world, this complication is a concern to the medical field. We report a case on the acute management of a patient who presented to us with life-threatening bilateral pulmonary embolism as a complication of VITT after the first dose of vaccination with Oxford-AstraZeneca vaccine against COVID-19.

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.

Thrombotic thrombocytopenic purpura following administration of the Moderna booster vaccine.

Thrombotic thrombocytopenic purpura (TTP) is a type of thrombotic microangiopathy that is characterized by microangiopathic haemolytic anaemia, consumption thrombocytopenia and organ injury. It is caused by a severe deficiency of ADAMTS13, which can be either congenital or acquired. There is a plethora of things that can cause the acquired form, including medications and infections. Vaccines have also been shown to cause TTP. In the midst of the COVID-19 pandemic, with multiple new vaccines being developed and distributed to the masses, the medical community needs to be aware of adverse events associated with these new vaccines. We present a case of TTP following administration of the Moderna booster vaccine.

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.

Case of acquired haemophilia a in Southeast Asia following COVID-19 vaccine.

Acquired haemophilia A (AHA) is a rare bleeding disorder with high morbidity and mortality, but it is eminently treatable if diagnosis and treatment are prompt. We report a case of AHA in Southeast Asia following the administration of the Pfizer-BioNTech COVID-19 vaccine. A man in his 80s developed multiple bruises 2 weeks after his first dose of the COVID-19 vaccine. Diagnosis was delayed due to his cognitive impairment and low clinical suspicion. This led to a representation with worsening ecchymosis, a left thigh haematoma and symptomatic anaemia. Laboratory testing was notable for an isolated prolongation of the activated partial thromboplastin time, which remained uncorrected in the mixing test. Further testing confirmed the presence of factor VIII (FVIII) inhibitors and low FVIII titres of 6.7%. He responded to treatment with intravenous methylprednisolone and recombinant activated FVII. Screening for autoimmune diseases and malignancies was negative.

Multiterritorial strokes in the setting of spontaneous heparin-induced thrombocytopaenia syndrome.

We present a case study of a 38-year-old man who developed arterial and venous thrombi, resulting in multiterritorial strokes, a pulmonary embolus and a cerebral venous sinus thrombosis in the setting of spontaneous heparin-induced thrombocytopaenia syndrome.

Acute respiratory distress syndrome precipitated by granulocyte colony-stimulating factor in undiagnosed Pneumocystis jirovecii pneumonia.

We present the case of a 62-year-old man with rheumatoid arthritis who developed a leukaemoid reaction and acute respiratory distress syndrome (ARDS) following granulocyte colony-stimulating factor (G-CSF) administration that had been given to treat neutropenia secondary to methotrexate and leflunomide toxicity. Later it was established that he had Pneumocystis jirovecii pneumonia, which was treated to complete resolution with a course of corticosteroids and antibiotics. This case highlights the potential risk of G-CSF administration in an immune compromised individual in the midst of bone marrow recovery in the context of active infection. Recognition of immune escape syndromes is vital and requires an understanding of potential triggers and risk factors.

Postoperative fever secondary to enoxaparin usage with pork allergy.

Postoperative fevers are common in hospitalised patients and warrant workup beyond the early post-op period. A 50-year-old man was admitted after sustaining a tibial plateau fracture. Fevers began 3 days after external fixation and persisted through a second surgery despite initial negative workup. Careful review of medications revealed enoxaparin as the instigating agent of a febrile drug reaction, and the fevers resolved after discontinuing the drug. On further questioning, it was discovered the patient had an allergy to pork, from which the main components of enoxaparin are typically derived. To our knowledge, this is the first reported enoxaparin-induced fever in the setting of a pork allergy. Enoxaparin-induced fevers should be considered in patients with unexplained post-op fever. Our case demonstrates the importance of analysing newly administered medications. Simple detailed history may significantly reduce patient morbidity and help to broaden differentials during investigation.

Haemophagocytic lymphohistiocytosis (HLH)-associated stress cardiomyopathy secondary to autoimmune conditions successfully treated with anakinra.

We describe two young cases of reactive haemophagocytic lymphohistiocytosis (HLH) with the resultant stress cardiomyopathy in the setting of underlying autoimmune diseases, systemic lupus erythematosus (SLE) and Still's disease. The initial presentation was similar in both cases with fever, hyperinflammatory response, hypotension (vasoplegia), bicytopenia and hyperferritinemia. Despite standard of care and multiple broad-spectrum antibiotics, both cases remained pyrexic and were ultimately admitted to the intensive therapy unit to treat cardiogenic shock. Echocardiogram of both cases showed low ejection fraction, the cause for which was not found until the final diagnosis of HLH was made. Both cases made a complete clinical and cardiac recovery following the initiation of high-dose glucocorticoids and anakinra.

Self-limiting severe neutropenia in a patient with COVID-19.

Neutropenia is a rare haematological complication of COVID-19 infection in immunocompetent patients. There is sparse literature on neutropenia in patients with COVID-19, except a few case reports. We encountered a similar case in an intensive care unit that developed severe neutropenia on day 24 of illness. Neutropenia resolved spontaneously on 4th day of its appearance. The patient was isolated and kept under close observation, antibiotics were upgraded and strict asepsis was maintained. Thus, we observed in a patient with no comorbidities and uncomplicated neutropenia that strict measures to prevent infection may suffice and the undue risk of hematopoietic therapy can be avoided. An expert opinion should always be sought in such cases as the presence of complications may require an aggressive approach.

Nitrofurantoin-induced agranulocytosis.

Idiosyncratic drug-induced agranulocytosis is a rare life-threatening adverse reaction characterised by an absolute neutrophil count <500 cells/µL of blood. Nitrofurantoin has been associated with haematological adverse events, but few agranulocytosis cases worldwide have been reported. We present a case of a 68-year-old woman who presented with fever and agranulocytosis following treatment with nitrofurantoin. Extensive workup for agranulocytosis, including a bone marrow aspirate, was unremarkable. Treatment with nitrofurantoin was discontinued, which led to a complete recovery of the complete blood count. This case stresses the importance of monitoring treatments, given that widely used drugs are not free from severe adverse reactions.

Immune thrombocytopenic purpura secondary to SARS-CoV-2 infection in a child with acute lymphoblastic leukaemia: a case report and review of literature.

Immune thrombocytopenic purpura (ITP) is characterised by isolated thrombocytopenia which may be idiopathic or due to a secondary aetiology. ITP is being increasingly recognised secondary to SARS-CoV-2 infection in the current pandemic. Here, we report a case of a five-and-a-half-year-old female child on maintenance chemotherapy for acute lymphoblastic leukaemia who subsequently developed ITP secondary to SARS-CoV-2 infection. Our patient had prolonged thrombocytopenia secondary to ITP, requiring the use of second-line agents including romiplostim and eltrombopag. This is a unique case where ITP was recognised secondary to SARS-CoV-2. In such cases of thrombocytopenia, ITP should be considered as an important differential in addition to relapse of leukaemia or thrombocytopenia due to chemotherapy drugs.

Complicated and persistent severe COVID-19 pneumonia in a recipient of allogeneic haematopoietic stem cell transplant.

We describe the case of a 45-year-old man affected by T-cell acute lymphoblastic leukaemia and diagnosed with COVID-19 early after an allogeneic haematopoietic stem cell transplant. The infectious disease was characterised by a severe and prolonged course, further complicated by a spontaneous pneumomediastinum and pneumopericardium. We successfully treated this patient with the antiviral drug remdesivir associated with two courses of COVID-19 convalescent plasma. This case report represents a good example of the typical clinical course of COVID-19 in severely immunosuppressed patients and gives evidence that in this population only a prompt treatment directed towards viral clearance can face the absence of a valid immune reactivity.

Haematuria, a widespread petechial rash, and headaches following the Oxford AstraZeneca ChAdOx1 nCoV-19 Vaccination.

With increasing presentations of headaches following COVID-19 vaccination, we present one of the UK's earliest proven cases of vaccine-induced thrombotic thrombocytopaenia (VITT), with the aim of giving colleagues a case to compare other patients against. Our patient was a 48-year-old man who presented with frank haematuria, a widespread petechial rash, and headaches, 2 weeks after receiving the first dose of the Oxford AstraZeneca ChAdOx1 nCoV-19 vaccine. He had a platelet count of 14×109/L and an extensive cerebral venous sinus thrombosis (CVST) with subarachnoid haemorrhage on imaging. He developed localising neurological signs and experienced a cardiopulmonary arrest. He was successfully resuscitated and transferred to a tertiary care centre for urgent thrombectomy. This case illustrates how the diagnosis of VITT should be based on the platelet count and imaging-and how patients with VITT should be cared for in centres with urgent neurosurgical and interventional radiology services.

Doxycycline-induced acquired haemophilia A.

An 80-year-old man with no personal or family history of bleeding, presented to hospital with extensive haematomas and skin bruising after using doxycycline. His basic lab workup was concerning for a coagulopathy with an elevated activated partial thromboplastin time and significant anaemia. Mixing studies and other factor levels were tested that led to the diagnosis of acquired haemophilia A with low factor VIII levels and high factor VIII antibodies. He was started on steroids, but his haemoglobin level continued to drop. Later, during his treatment, he was given multiple therapeutic agents, including cyclophosphamide, rituximab and recombinant factor VII (NovoSeven-R). Gradually factor VIII levels increased and haemoglobin stabilised. The hospital course was complicated by COVID-19 pneumonia leading to acute respiratory distress syndrome; the patient eventually expired due to respiratory failure.