Thrombotic Thrombocytopenic Purpura Without Schistocytes: Beware of Misdiagnosis

2021 ◽  
pp. 10.1212/CPJ.0000000000001067
Author(s):  
P. Decker ◽  
T. Moulinet ◽  
S. Revuz ◽  
P. Perez ◽  
R. Jaussaud

A 43-year old woman was admitted for acute unusual headache, vertigo and left upper limb weakness. She had been complaining of asthenia for the past few weeks. Past medical history included uterine cervix cancer treated with conisation and two pregnancies without serious complications. No allergy or recent drug introduction was noted. After ruling out current pregnancy, Magnetic Resonance Imaging (MRI) was immediately performed and showed recent ischemic strokes in multiple territories. Doppler ultrasound imaging showed no carotid or vertebral arteries lesion. Electrocardiogram showed no ST elevation or atrial fibrillation and echocardiography did not reveal embolic lesion or patent foramen ovale. Blood cell count showed thrombocytopenia at 17 x 109/L (confirmed in citrate tube) and hyperleukocytosis at 18 x 109/L without anemia (hemoglobin at 12,9 g/dL). Basic metabolic profile, kidney and liver function tests were normal. Hemolysis signs were present: reticulocytes, total bilirubin, Lactate dehydrogenase (LDH) and haptoglobin levels were at 147 x 109/L, 27,2 μmol/L, 454 U/L and bellow 0.08 g/L respectively. Repeated blood smears showed no schistocytes. A disseminated intravascular coagulation was ruled out (normal APTT and prothrombin time, fibrinogen at 3,6 g/L), as well as autoimmune cytopenia (Coombs test was negative), hyperhomocysteinemia, vitamin deficiencies (B9, B12) and paroxysmal nocturnal hemoglobinuria. Bone marrow examination showed increased megakaryocytes with normal morphology excluding a central origin. Lupus anticoagulant (LA), anticardiolipin (aCL) and anti- β2gp1 antibodies were negative. A thoraco-abdominopelvic scan with contrast showed small renal and splenic infarcts; no suspect tumor was observed.

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Badrul Islam ◽  
Mohiuddin Ahmed ◽  
Zhahirul Islam ◽  
S. M. Begum

Abstract Background SARS-CoV2 virus could be potentially myopathic. Serum creatinine phosphokinase (CPK) is frequently found elevated in severe SARS-CoV2 infection, which indicates skeletal muscle damage precipitating limb weakness or even ventilatory failure. Case presentation We addressed such a patient in his forties presented with features of severe SARS-CoV2 pneumonia and high serum CPK. He developed severe sepsis and acute respiratory distress syndrome (ARDS) and received intravenous high dose corticosteroid and tocilizumab to counter SARS-CoV2 associated cytokine surge. After 10 days of mechanical ventilation (MV), weaning was unsuccessful albeit apparently clear lung fields, having additionally severe and symmetric limb muscle weakness. Ancillary investigations in addition with serum CPK, including electromyogram, muscle biopsy, and muscle magnetic resonance imaging (MRI) suggested acute myopathy possibly due to skeletal myositis. Conclusion We wish to stress that myopathogenic medication in SARS-CoV2 pneumonia should be used with caution. Additionally, serum CPK could be a potential marker to predict respiratory failure in SARS-CoV2 pneumonia as skeletal myopathy affecting chest muscles may contribute ventilatory failure on top of oxygenation failure due to SARS-CoV2 pneumonia.


2021 ◽  
Vol 12 (9) ◽  
pp. 79-83
Author(s):  
Noorin Zaidi ◽  
Rasha Zia Usmani ◽  
Kshama Tiwari ◽  
Sumaiya Irfan ◽  
Syed Riaz Mehdi

Background: There is a need to differentiate megaloblastic anemia from mixed deficiency anemia as both require different management protocols. With the acquisition of more information about them, tests such as serum vitamin estimation and Schilling test, were found to have their limitations. Hence there is a need to search newer diagnostic candidates to differentiate between megaloblastic anemia and mixed deficiency anemia. Aims and Objective: The current study was undertaken to find usefulness of serum Lactate Dehydrogenase (LDH) in differentiating megaloblastic anemia from mixed deficiency anemia. Materials and Methods: 100 patients were included in the study. Blood smears were stained and analysed. Complete blood counts were performed. Bone marrow examination was done, where needed. Biochemical tests were performed for estimation of vitamin B12, Folate and for LDH. Results: Out of the 100 cases 51 were diagnosed as megaloblastic anemia and 49 were diagnosed as mixed deficiency anemia. The LDH levels were significantly higher in cases of megaloblastic anemia as compared to mixed deficiency anemia. Conclusion: Serum LDH levels can be used in differentiating megaloblastic anemia from mixed deficiency anemia.


2009 ◽  
Vol 161 (suppl_1) ◽  
pp. S19-S24 ◽  
Author(s):  
Peter J Trainer

ACROSTUDY is an observational registry intended to collect safety and efficacy data on pegvisomant therapy. A total of 792 patients have been enrolled, of whom 83% had commenced pegvisomant prior to recruitment. The mean follow-up is 1.66 years with the mean duration of pegvisomant therapy 3.31 years representing 2625 patient years of treatment. About 90% of patients were on once daily pegvisomant, and 67% were on monotherapy. Disappointingly, IGF1 was normalised in <70% of patients; furthermore, in 80% of patients with an elevated IGF1, the daily dose of pegvisomant was 20 mg or less. A total of 56 serious adverse events (AEs) were reported, of which 13 were related to pegvisomant. A total of 276 AEs were reported, of which 56 were considered related to pegvisomant. The AEs most frequently attributed to pegvisomant were disturbed liver function tests and injection site reactions. Magnetic resonance imaging (MRI) was available in 684 patients. A total of 411 patients had at least one MRI on pegvisomant compared with a baseline. In 31 patients, a decrease in tumour size has been reported, of whom 20 had previously received radiotherapy. An increase in tumour size has been reported and confirmed in 22 patients. In 11 patients, there was contradictory data on tumour size, while, in six patients, central review of the films failed to confirm increase in tumour size. In conclusion, the safety data are generally reassuring, while the IGF1 normalisation rate is disappointing, which probably reflects a failure of dose titration. Further effort is needed to understand the reasons for the failure of dose titration.


2021 ◽  
Author(s):  
Carlos Eduardo Dilen da Silva ◽  
Allan Rubens Zucolloto ◽  
Bianca Castoldi Scuassante ◽  
Luciano Stefanato Negrini

Context: Dizziness or vertigo is a very common clinical manifestation, usually of labyrinthic origin. However, an accurated neurological clinical examination is crucial for the diagnosis of central origin. Case report: D.F.A, 40 years old, female, white, Brazilian. She has been reportining of intense dizziness for a few months and relevant postural instability. She reports two previous hospitalizations, without a closed diagnosis, and is then referred to the specialist. On clinical examination, she had bilateral internuclear ophthalmoparesis and bidirectional nystagmus in her right eye. Requested magnetic resonance imaging (MRI) of the skull suggestive of demyelinating lesions in the wall of the fourth ventricle, pons and, especially, in transition between medulla oblongata and spinal cord, compatible with Neuromyelitis Optica (NMO) with manifestations of trunk syndrome. After pulse therapy, she obtained a good clinical evolution, with the maintenance of sensory deficits as a result of the injury, but with significant improvement in bilateral ophthalmoparesis and balance. Some studies already report brainstem involvement in patients with NMO, describing the presence of brainstem´s signals. Conclusion: In the case in question, therefore, the patient remained for almost two months without an accurate diagnosis, being the neurological examination the crucial factor for the suggestion of a disease of central origin, reporting a case of onset NMO with of brainstem´s signals.


Author(s):  
Seyed A. Hosseini ◽  
Mohammad H. Gharib ◽  
Seyed B. Mirheidari ◽  
Bahar Ghanbarzade ◽  
Parnian S. Hosseini

AbstractAcute ischemic stroke is rare in children and often brings enduring and permanent morbidity in pediatric population. While there are numerous recommendations for initial treatment, evidence on effectiveness of different therapies among children is limited. Here, we describe a 10-month-old boy who presented with fever, restlessness, seizure and postictal left limb weakness. Examination revealed left hemiplegia and hemiparesthesia. Computed tomography (CT) head showed loss of gray–white matter distinction with diffuse hypodensity in the right temporoparietal lobes and right caudate nucleus. Magnetic resonance imaging (MRI) brain demonstrated diffusion restriction in favor of acute ischemic stroke in the right middle cerebral artery (MCA) territory. Brain MR angiography (MRA) demonstrated complete occlusion of proximal aspect of M1 segment of right MCA. Genetic testing determined a homozygous 4G/4G polymorphism of the PAI-1 gene. Antiplatelet therapy was started after diagnosis and continued for 8 days with antibiotic therapy. Stroke in children and infants is an infrequent condition associated with substantial morbidity and mortality that needs clinicians' care. This case highlights the significance of awareness about stroke in children and emphasizes on further research to compile evidence-based guidelines for acute stroke therapy in children.


2020 ◽  
Vol 6 (1) ◽  
pp. 41-48
Author(s):  
Deepak Garg ◽  
◽  
Gaurav Jain ◽  
Virendra Sinha ◽  
◽  
...  

Background and Importance: Hydatid cyst is endemic in India, with different clinical presentations according to the site of involvement in the body. However, its occurrence in the brain without the involvement of other body parts is rare. Case Presentation: An eight-year-old female child presented with right-sided limb weakness and slurring of speech. The diagnosis of primary brain hydatid cyst was made with Magnetic Resonance Imaging (MRI) of the brain. The cyst was removed completely, resulting in improved post-operative slurring of speech and limb weakness. Conclusion: In countries where hydatid disease is endemic, brain hydatidosis should on top of the differential diagnoses for intracerebral cystic lesions and managed as per hydatid protocol until proved otherwise. This approach is critical to prevent rupture and dissemination.


BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Wee Yong Tan ◽  
Abdul Hanif Khan Yusof Khan ◽  
Mohd Naim Mohd Yaakob ◽  
Anna Misyail Abdul Rashid ◽  
Wei Chao Loh ◽  
...  

Abstract Background Transverse myelitis (TM) is a relatively uncommon condition, and vaccine-associated myelitis is even rarer. Concern regarding neurological complications following vaccination escalated following the report of TM during the safety and efficacy trials of the COVID-19 vaccine. Case presentation We report the first case of Longitudinal Extensive Transverse Myelitis (LETM) in Malaysia following administration of the chimpanzee adenovirus-vectored (ChAdOx1 nCoV-19) vaccine. A 25-year-old female presented with bilateral lower limb weakness and inability to walk with a sensory level up to T8 with absent visual symptoms. Urgent gadolinium-enhanced magnetic resonance imaging (MRI) of the spine showed long segment TM over the thoracic region. Cerebrospinal fluid autoantibodies for anti-aquaporin-4 and anti-myelin-oligodendrocyte were negative. A diagnosis of LETM following vaccination was made, and the patient was started on a high dose of intravenous methylprednisolone. The patient eventually made a recovery following treatment. Conclusion LETM is a rare but serious adverse reaction following vaccination. Previously reported cases showed an onset of symptoms between 10 to 14 days post-vaccination, suggesting a delayed immunogenic reaction. However, the incidence of myelitis in COVID-19 is much more common, far greater than the risk associated with vaccination.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Ken C Oba ◽  
Udaya M Kabadi

Abstract Background: Short bowel syndrome is characterized by maldigestion and malabsrption resulting in deficiencies of multiple nutrients including vitamins and minerals. Most subjects required parental elimination for survival. GLP 2 RA Teduglutide was recently approved for treatment of short-bowel syndrome especially for those requiring parenteral support. Objective: To demonstrate the utility of GLP2 Receptor Agonist Teduglutide in improvising multiple metabolic indices In presence of short bowel syndrome. Case presentation: 66-year-old Caucasian female presented with a history of short bowel syndrome and associated vitamin deficiencies, hypothyroidism requiring large dose of levothyroxine, diarrhea, and liver cirrhosis. Upon starting teduglutide the subject saw improvement in her symptoms. Moreover, daily dose of levothyroxine required reductions from 300 mcg to 150 mcg to maintain desirable serum concentrations of free T4, free T3 and TSH. Finally, serum levels of several vitamins attained greater than therapeutic concentrations requiring dosage reductions. Also notable was the improvement in her liver function tests, remission from ascites and regeneration of liver nodules. Conclusion: Herein, we report an adult subject with short bowel syndrome with concurrent hypothyroidism and multiple vitamin deficiencies who following administration of GLP2 RA therapy demonstrated a marked improvement in her metabolic parameters with some requiring reduction in daily dose along with improvement in manifestations of liver cirrhosis.


2021 ◽  
Vol 93 (4) ◽  
pp. 376-380
Author(s):  
Ekaterina S. Pershina ◽  
Dmitry Yu. Shchekochikhin ◽  
Georgii M. Shaginyan ◽  
Alexandra S. Shilova ◽  
Andrei V. Sherashov ◽  
...  

Aim. To analyze diagnostic performance of cardiovascular magnetic resonance (CMR) in patients, presented with myocardial infarction with nonobstructed coronary arteries (MINOCA). Materials ant methods. 46 consecutives patients presented with myocardial infarction without evidence of obstructive coronary disease on angiography between January, 1 2018 and October 1, 2019 were included in the study. All patients underwent CMR within 10 days after admission. MRI was performed on 1.5 T Magnetic Resonance Imaging (MRI) using comprehensive protocol (T2-images, Cine-CMR, late gadolinium enhancement (LGE)). Results. CMR revealed myocardial infarction (MI) pattern in 14 patients (30.4%), myocarditis in 12 (26.1%), hypertrophic cardiomyopathy in 6 (13.1%). In 14 patients (30.4%) no LGE was observed. Notably in 2 patients without LGE features of takotsubo syndrome were noted. Mean age was significantly lower in patients with MI versus patient with non-ischemic causes of MINOCA (56.112.3 vs 64.612.8; p=0.04). ST elevation at admission frequency didnt differ between MI and non-ischemic patients (35.7% vs 25.0%; p=0.76). However MI patients had significantly increased troponin level, 0.87 [0.22; 1.85] vs 0.22 [0.07; 0.38]; p=0.008. CMR allowed to establish the prcised clinical diagnosis in 73.9% of the cases. Conclusion. Clinical data doesnt allow to differentiate ischemic or non-ischemic causes of MINOCA. However, CMR establish the correct diagnosis in most cases.


2020 ◽  
Vol 17 (3) ◽  
pp. 75-80
Author(s):  
Zafar Hayat ◽  
Inam Ullah ◽  
Khizar Hayat ◽  
Hashimuddin Azam Khan ◽  
Fazle Raziq ◽  
...  

Background: Falciparum malaria is a common disease in our area. Apart from its classical presentation, at times it may present with refractory anemia or pancytopenia. The aim of this study was to determine the refractory anemia and pancytopenia as complications of falciparum malaria and apart from peripheral blood smears the significance of rapid antigen tests and bone marrow examination in the diagnosis of falciparum malaria. Material & Methods: The descriptive study included 200 consecutive cases of fever and refractory anemia or pancytopenia from 2011 to 2014. Stratification of patients according to the clinical scenario included Group-A having fever with refractory anemia and Group-B with fever and pancytopenia. A detailed history, thorough clinical examination, and pertinent laboratory tests were performed. All patients were treated with antimalarial drugs and followed-up for eight weeks. The pre and post treatment hematologic parameters were compared. Results: Among the 200 patients, 85 were males and 115 females. The age ranged from 15 to 55 years. Stratification of patients on clinical scenario revealed 175(87.5%) patients with fever and refractory anemia (Group-A). Among these, 125(62.5%) patients were reported smear positive for P. falciparum. In the remaining 50 smear negative patients rapid antigen tests were performed and all were reported positive. In 25 patients of Group B with fever and pancytopenia, the peripheral smear for malaria was positive only in 5 patients. In the remaining 20 cases both the peripheral blood smears and rapid antigen tests were reported negative. Bone marrow examination was planned to confirm the bone marrow suppression as the cause of peripheral pancytopenia, to exclude leukemia and to identify P. falciparum. The bone marrow examination revealed P. falciparum in all these cases. All the patients had a dramatic response to treatment with antimalarials in terms of disappearance of fever and correction of anemia and bone marrow rescue with reversal of pancytopenia to normal counts. Conclusion: Plasmodium falciparum should be considered in all cases of prolonged fever with refractory anemia or pancytopenia in malaria endemic areas, even with negative smear and rapid antigen tests. Bone examination is mandatory for the diagnosis in such cases. There is dramatic response of such patients to treatment with antimalarial drugs and hematinics.


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