One Haemolytic Anaemia May Hide Another: Paroxysmal Nocturnal Haemoglobinuria Masquerading As Plasmodium Falciparum Infection.

Background: Paroxysmal nocturnal haemoglobinuria (PNH) is a rare, genetic and acquired haematologic disease that causes complement-mediated intravascular haemolytic anaemia, thrombosis and bone marrow failure. Case description: A 27-year-old migrant patient attended the emergency department in a context of fever and chills over the previous few days as well as chronic fatigue, dyspnoea and chest pain. His medical history included chronic anaemia and erectile dysfunction. Initial biology showed a haemoglobin of 6.3 g/dl, platelets of 25,000/µl, total leucocytes of 3,500/µl with 1,500 neutrophils. B12 vitamin, folic acid, ferritin and thyroid stimulating hormone were normal. Lactate dehydrogenase levels were high and haptoglobin was non-measurable. C-reactive protein was 46.1 mg/l. A thick blood smear revealed Plasmodium falciparum infection with 0.1% parasitaemia. The patient was treated with an oral combination of artemether and lumefantrine. Three weeks later, the patient consulted the infectious disease department given the lack of clinical improvement. The cytopenias worsened, and lactate dehydrogenase (LDH) and reticulocytes increased. Tests for schistocytes, a thick blood smear for malaria and a direct Coombs test were negative; a myelogram was reassuring. An abdominal, pelvic and thoracic CT scan showed a mild hepatomegaly with no focal lesion and no splenomegaly or adenomegaly. A 12-colour flow cytometry unveiled a PNH clone on 90.9545% of neutrophils and 80.7371% of monocytes. Discussion: PNH patients can be vulnerable to parasites infection (such as P. falciparum) as it may trigger breakthrough haemolysis through uncontrolled resurgence of activity of the complement system. In our patient, P. falciparum infection was a confounding factor, as it commonly causes haemolytic anaemia and thrombocytopenia, and patients living in malaria-endemic regions can carry low parasitaemia while being slightly symptomatic or asymptomatic. LEARNING POINTS: Plasmodium falciparum infection can cause breakthrough haemolysis in patients with paroxysmal nocturnal haemoglobinuria.Low P. falciparum parasitemia in patients living in malaria-endemic regions is not always significant as these patients often carry acquired immunity.Patients from malaria-endemic regions presenting with severe sickness and low P. falciparum parasitemia must be assessed for other diseases, as it cannot explain heavy illness.Patients presenting with haemolytic anaemia, no schistocytes, a negative direct Coombs test and other unexplained cytopenia such as thrombocytopenia/neutropenia and other unexplained clinical manifestations such as dyspnoea, chest pain or erectile dysfunction should be assessed for paroxysmal nocturnal haemoglobinuria.

The spectrum of glomerular and vascular kidney pathology associated with myeloproliferative neoplasms.

A high prevalence of chronic kidney disease (CKD) occurs in patients with myeloproliferative neoplasms (MPN). However, MPN-related glomerulopathy (MPN-RG) may not account for the entirety of CKD risk in this population. The systemic vasculopathy encountered in these patients raises the hypothesis that vascular nephrosclerosis may be a common pattern of injury in patients with MPN and with CKD. In an exhaustive, retrospective, multicenter study of MPN kidney biopsies in four different pathology departments, we now describe glomerular and vascular lesions and establish clinicopathologic correlations. Our study encompassed 47 patients with MPN who underwent a kidney biopsy that included 16 patients with chronic myeloid leukemia (CML) and 31 patients with non-CML MPN. Fourteen cases met a proposed definition of MPN-RG based on mesangial sclerosis and hypercellularity, as well as glomerular thrombotic microangiopathy. MPN-RG was significantly associated with both myelofibrosis and poorer kidney survival. Thirty-three patients had moderate-to-severe arteriosclerosis while 39 patients had moderate-to-severe arteriolar hyalinosis. Multivariable models that included 188 adult native kidney biopsies as controls revealed an association between MPN and chronic kidney vascular damage, which was independent of established risk factors such as age, diabetes mellitus and hypertension. Therefore, MPN-RG is associated with myelofibrosis and has a poor kidney prognosis. Thus, our findings suggest that the kidney vasculature is a target during MPN-associated vasculopathy and establish a new link between MPN and CKD. Hence, these results may raise new hypotheses regarding the pathophysiology of vascular nephrosclerosis in the general population.

Disseminated Nocardia transvalensis complex and farcinica: First case in an immunocompetent.

Background: Nocardiae is an opportunistic infection mainly of the immunocompromised patient without sparing the immunocompetent subject or without any identified risk factors. They can be localized or disseminated. The extreme rarity of this infection often results in a deleterious diagnostic delay. Case presentation: We report a first case of community acquired pneumonia with asymptomatic disseminated brain abscess due to Nocardia transvalensis/wallacei and farcinica in an immunocompetent man. The patient fully recovered after receiving optimized antimicrobial therapy. Conclusions: This case suggests that health care professionals should always evoke this diagnosis when confronted to atypical community-acquired pneumonia, even in immunocompetent patients.

A Fatal Case Coronavirus Disease 2019 - Associated Acute Hemorrhagic Necrotizing Encephalopathy.

Coronavirus disease 2019 (COVID-19) has been reported in association with a variety of brain imaging findings such as acute hemorrhagic necrotizing encephalopathy. To the best of our knowledge, we are reporting a second case of acute necrotizing hemorrhagic encephalopathy associated with COVID-19, which was fatal in a few hours in a 56-year-old male without a specific history. We claim that this case is important because this case shows that the unconscious patients are potentially infected by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and might cause the horizontal infection. In order to end the pandemic of SARS-CoV-2 diseases, the diagnosis of the disease must be prompt and not overlook any findings. We think that diffusion magnetic resonance imaging is a promising and useful sequence to evaluate the changes in brain tissue in the acute necrotizing encephalopathy.

Acute disseminated encephalomyelitis after dengue.

Dengue fever, transmitted by Aedes aegypti mosquitoes, is one of the most common vector-borne disease. Its incidence is increasing steadily worldwide, becoming a major public health problem in the tropical and subtropical zone. Neurological manifestations after dengue are not very common and acute disseminated encephalomyelitis (ADEM) following dengue infections is rare with a few cases documented in literature. Clinical characteristics and typical lesions of ADEM on magnetic resonance imaging (MRI) of brain along with serologic positivity for dengue usually confirm the diagnosis. We report a case of ADEM which developed as a neurological complication of dengue during an epidemic in a 39-year-old woman.