2,3-Diphosphoglycerate Concentrations in Autologous Salvaged Versus Stored Red Blood Cells and in Surgical Patients After Transfusion.
Anesthesia and analgesia
BACKGROUND:Stored red blood cells (RBCs) are deficient in 2,3-diphosphoglycerate (2,3-DPG), but it is unclear how autologous salvaged blood (ASB) compares with stored blood and how rapidly 2,3-DPG levels return to normal after transfusion. Therefore, we compared levels of 2,3-DPG in stored versus ASB RBCs and in patients' blood after transfusion. METHODS:Twenty-four patients undergoing multilevel spine fusion surgery were enrolled. We measured 2,3-DPG and the oxyhemoglobin dissociation curve (P50) in samples taken from the ASB and stored blood bags before transfusion and in blood samples drawn from patients before and after transfusion. RESULTS:The mean storage duration for stored RBCs was 24 ± 8 days. Compared with fresh RBCs, stored RBCs had decreased 2,3-DPG levels (by approximately 90%; P < 0.0001) and a decreased P50 (by approximately 30%; P < 0.0001). However, ASB RBCs did not exhibit these changes. The mean 2,3-DPG concentration decreased by approximately 20% (P < 0.05) in postoperative blood sampled from patients who received 1 to 3 stored RBC units and by approximately 30% (P < 0.01) in those who received ≥4 stored RBC units. 2,3-DPG was unchanged in patients who received no stored blood or ASB alone. After surgery, 2,3-DPG levels recovered gradually over 3 postoperative days in patients who received stored RBCs. CONCLUSIONS:Stored RBCs, but not ASB RBCs, have decreased levels of 2,3-DPG and a left-shift in the oxyhemoglobin dissociation curve. Postoperatively, 2,3-DPG levels remain below preoperative baseline levels for up to 3 postoperative days in patients who receive stored RBCs but are unchanged in those who receive only ASB RBCs.
10.1213/ANE.0000000000001071
Renal recovery with eculizumab in atypical hemolytic uremic syndrome following prolonged dialysis.
Povey Hannah,Vundru Rahul,Junglee Naushad,Jibani Mahdi
Clinical nephrology
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) which encompasses hemolytic anemia, thrombocytopenia, and organ impairment. Around 10% of cases are atypical HUS (aHUS), a rare disease with poor outcomes caused by uncontrolled activation of the alternative complement pathway. This case describes a young woman with clinical manifestations compatible with TMA during childhood and adolescence who was formally diagnosed with aHUS at the age of 21. She was managed with intensive plasma exchange and hemodialysis, which failed to improve her severe acute kidney injury and other hematological manifestations of aHUS. This was further compounded by several episodes of flash pulmonary edema and the posterior reversible encephalopathy syndrome (PRES). Treatment with the monoclonal anti-C5 inhibitor, eculizumab, improved all hematological parameters with almost full renal recovery following 3.5 months of dialysis. So far, long-term use of eculizumab (> 11 months) continues to be effective and without complication. Our case illustrates the difficulty but importance of early consideration of aHUS in patients presenting with TMA. More importantly, we highlight that near-normal renal recovery may be attained with eculizumab in adults even after a long dependence on dialysis - an observation that has not been reported in the literature so far.
10.5414/CN107958
Reversible posterior leukoencephalopathy syndrome: a possible manifestation of Wegener's granulomatosis-mediated endothelial injury.
Nishio Minako,Yoshioka Katsunobu,Yamagami Keiko,Morikawa Takashi,Konishi Yoshio,Hayashi Noriko,Himuro Kimihide,Imanishi Masahito
Modern rheumatology
We present the case of a 15-year-old girl who had Wegener's granulomatosis with severe intestinal involvement. During the clinical course, she developed generalized seizures and was diagnosed with reversible posterior leukoencephalopathy syndrome (RPLS). Plasma exchange combined with steroid pulse therapy was initiated and showed marked improvement. This is one of the few cases of RPLS without severe hypertension or renal failure, suggesting that RPLS is likely to be a manifestation of Wegener's granulomatosis-mediated endothelial injury.
10.1007/s10165-008-0052-1
Thrombotic thrombocytopenic purpura presenting with reversible posterior leukoencephalopathy syndrome.
AOTA Yasuo,KODAMA Shuichi,KITAGAWA Naoyuki,KAWABATA Sohya,GOTOH Akihiko,SAKURAI Michio
[Rinsho ketsueki] The Japanese journal of clinical hematology
A 48-year-old man presented with consciousness disturbance with vasogenic edema in the occipital lobe on brain CT. The diagnosis of reversible posterior leukoencephalopathy syndrome (RPLS) was made. His hypertension was refractory to treatment, and his neurological disabilities and CT abnormalities, along with renal dysfunction, became worse. Hemodialysis and strict management of blood pressure resolved the neurological findings and the lesions on brain CT. However, one week later, consciousness disturbance and brain CT abnormalities recurred. At that time, hemolytic anemia with fragmented erythrocytes, thrombocytopenia, and renal dysfunction became apparent. We therefore diagnosed thrombotic thrombocytopenic purpura (TTP). Plasma exchange and vincristine administration improved not only the clinical findings of TTP, but also consciousness disturbance and brain CT abnormalities. We concluded that latent TTP had caused RPLS in this patient.
Neurological PRESentations in Sickle Cell Patients Are Not Always Stroke: A Review of Posterior Reversible Encephalopathy Syndrome in Sickle Cell Disease.
Solh Ziad,Taccone Michael S,Marin Samantha,Athale Uma,Breakey Vicky R
Pediatric blood & cancer
Acute neurological changes in sickle cell disease (SCD) patients often raise the suspicion for stroke. Posterior reversible encephalopathy syndrome (PRES) can mimic stroke in its clinical presentation. We aimed to (i) review the PRES literature in SCD patients including clinical presentation, risk factors, pathophysiology, and management and (ii) elucidate the distinction between PRES and stroke in SCD. The exact pathophysiology of PRES in SCD remains elusive but is likely multifactorial and related to sickling, ischemia, and chronic anemia predisposing to vasogenic edema. PRES and stroke in SCD are distinguishable conditions. Our review may help elucidate a clinical approach to this distinction.
10.1002/pbc.25932
[Posterior reversible encephalopathy in a girl with systemic lupus erythematosus: Report of a case].
Marín Gustavo R
Archivos argentinos de pediatria
Posterior reversible encephalopathy is a rare disease in children. Clinical manifestations include headache, seizures, visual disturbances and altered consciousness associated with typical magnetic resonance images of the nervous system. The syndrome usually manifests in patients with eclampsia, solid organ transplantation, haematologic, renal and autoimmune diseases among other less common causes and it is often triggered after a hypertensive crisis or use of immunosuppressive drugs. Less common pathogenic factors as blood transfusion, use of immunoglobulins or an underlying infection can be associated. In this case a girl with systemic lupus erythematosus and exposed to multiple etiopathogenic factors developed posterior reversible encephalopathy.
10.5546/aap.2015.e271
[PRES: Posterior Reversible Encephalopathy Syndrome].
Okamoto Kouichirou,Motohashi Kunio,Fujiwara Hidemoto,Ishihara Tomohiko,Ninomiya Itaru,Onodera Osamu,Fujii Yukihiko
Brain and nerve = Shinkei kenkyu no shinpo
Posterior reversible encephalopathy syndrome (PRES) is suggested in patients with acute neurological symptoms in the appropriate clinical context, including acute hypertension, blood pressure fluctuations, renal failure, blood transfusion, immunosuppression, autoimmune disorders, and eclampsia. PRES is a clinical syndrome, and refers to a disorder with reversible subcortical vasogenic brain edema caused by endothelial dysfunction, predominantly involving the bilateral parieto-occipital regions. Although the clinical course and prognosis are favorable in most cases, intracranial hemorrhage and/or restricted diffusion similar to acute infarction could be seen in some lesions on brain magnetic resonance imaging (MRI). The spinal cord may be involved in some patients with posterior fossa lesions. Understanding the pathophysiology of PRES is helpful in making the correct early diagnosis and selecting appropriate therapies to improve its clinical course and outcome. Differentiation of PRES from strokes is critical in the setting of a neurological emergency.
10.11477/mf.1416200653
Reversible cerebral vasoconstriction syndrome after blood transfusion.
Dou Yi-Hsuan,Fuh Jong-Ling,Chen Shih-Pin,Wang Shuu-Jiun
Headache
OBJECTIVES:To report 2 cases of reversible cerebral vasoconstriction syndrome (RCVS) with posterior reversible encephalopathy syndrome (PRES) after blood transfusion for severe anemia. BACKGROUND:RCVS is presented with recurrent thunderclap headache and reversible constriction of cerebral arteries. PRES is a known complication of RCVS. Blood transfusion for severe anemia could be a cause for PRES in few cases; however, it is seldom mentioned as an etiology for RCVS. METHODS:We report a case series. RESULTS:We report 2 women presented with RCVS with PRES after blood transfusion for anemia, and reviewed another 4 similar cases reported in the literature. Our 2 patients were middle-aged women, with severe chronic anemia (average hemoglobin: 1.45 g/dL), and received multiple blood transfusions (average: 3250 mL) over a period of 5-7 days. They developed thunderclap headache and other symptoms about 1 week after the last blood transfusion. Cerebral vasoconstrictions were demonstrated by magnetic resonance angiography and transcranial color-coded sonography. PRES was found in both of them using magnetic resonance imaging, and one of them also had cytotoxic edema on diffusion weighted image. CONCLUSIONS:RCVS with PRES is one complication of blood transfusion in patients under chronic severe anemia (especially when hemoglobin level increased for more than 5 g/dL), particularly in Asian women with menorrhagia. Blood pressure surge and the occurrence of severe headaches or other neurological symptoms should be aggressively monitored within 10 days after the last blood transfusion.
10.1111/head.12319
Reversible posterior leukoencephalopathy syndrome after blood transfusion in a patient with end-stage renal disease.
Sato Yoshinori,Hirose Makoto,Inoue Yoshihiko,Komukai Daisuke,Takayasu Mamiko,Kawashima Eri,Koiwa Fumihiko,Yoshimura Ashio
Clinical and experimental nephrology
A 42-year-old female end-stage renal disease (ESRD) patient with reversible posterior leukoencephalopathy syndrome (RPLS) post-transfusion during initiation of hemodialysis is reported. Eleven days after the onset of illness, we diagnosed encephalopathy as a grand mal seizure resulting from diffuse cerebral edema. One reason for the delayed diagnosis was that her symptom, a throbbing headache that occurred during her first dialysis, indicated dialysis disequilibrium syndrome. We must bear in mind that a small amount of transfusion could cause RPLS even during the first dialysis. To our knowledge, this is the first case report on RPLS after blood transfusion in an ESRD patient.
10.1007/s10157-011-0515-0
Posterior Reversible Encephalopathy Syndrome in a Five-Year-Old Child: A Case Report.
Acar Sencan,Kavlak Mustafa Emre,Demir Baris,Ozkan Perihan,Polat Kamil Yalcin,Akyildiz Murat,Arikan Cigdem
Transplantation proceedings
Posterior reversible encephalopathy syndrome (PRES) is a neuroradiologic syndrome. The etiology of PRES is still unclear. Some factors were described. We present a case of a pediatric patient with liver transplant who developed PRES following blood transfusion while receiving tacrolimus therapy. A 5½-year-old boy who underwent living donor liver transplantation, and PRES developed on the sixth day post transplant under tacrolimus treatment after 6 hours of red blood transfusion. PRES is a rare condition; it should be kept in mind about patients who have received organ transplants and develop sudden neurologic symptoms.
10.1016/j.transproceed.2019.01.186
Reversible posterior leukoencephalopathy syndrome caused by blood transfusion: a case report.
Huang Yung-Chuan,Tsai Pei-Lin,Yeh Jiann-Horng,Chen Wei-Hung
Acta neurologica Taiwanica
This is a case report of a 32-year-old woman with chronic severe anemia who developed headaches and seizures 5 days after receiving a blood transfusion of eight units (1600 ml) of packed red blood cells. Magnetic resonance imaging indicated vasogenic edematous lesions bilaterally over the occipital lobes that were consistent with reversible posterior leukoencephalopathy syndrome (RPLS). Her blood pressure was normal, and no other contributing factors for RPLS were found. It is likely that the initiator was the large volume of transfused blood, which disrupted cerebral autoregulation and damaged the vasculoendothelial system. Similar cases of RPLS following transfusion have been reported, and all reports involved middle-aged females with chronic severe anemia who received large volumes of transfused blood within a short period of time. Although blood transfusion is a common procedure with rare neurological complications, great caution should be taken with chronic severely anemic patients because a rapid elevation in hemoglobin may precipitate RPLS.
Assessment of Noninvasive Regional Brain Oximetry in Posterior Reversible Encephalopathy Syndrome and Reversible Cerebral Vasoconstriction Syndrome.
Chung David Y,Claassen Jan,Agarwal Sachin,Schmidt J Michael,Mayer Stephan A
Journal of intensive care medicine
BACKGROUND:Posterior reversible encephalopathy syndrome (PRES) leads to small- and large-vessel circulatory dysfunction. While aggressive lowering of elevated blood pressure is the usual treatment for PRES, excessive blood pressure reduction may lead to ischemia or infarction, particularly when PRES is accompanied by reversible cerebral vasoconstriction syndrome (RCVS). Regional cerebral oximetry using near-infrared spectroscopy is a noninvasive modality that is commonly used intraoperatively and in intensive care settings to monitor regional cerebral oxygenation (rSO2) and may be useful in guiding treatment in select cases of PRES and RCVS. RESULTS:We report a case of a patient with PRES complicated by infarction and RCVS where the optimal blood pressure management was unclear. A decision was made to decrease blood pressure which resulted in an improved neurological examination and increase in rSO2 from 40% to 55% in at-risk brain. Infarcted brain as determined by diffusion-weighted magnetic resonance imaging and computed tomography perfusion imaging showed no change in rSO2 during the same time period. Furthermore, there was a qualitative change in the rSO2-mean arterial pressure (MAP) relationship, suggesting an alteration in cerebrovascular autoregulation as a result of lowering blood pressure. CONCLUSIONS:Regional cerebral oximetry can provide valuable diagnostic feedback in complicated cases of PRES and RCVS.
10.1177/0885066615623465
[Case of post-transfusion posterior reversible encephalopathy syndrome with cerebral hemorrhage that may be associated with fat-soluble vitamin deficiency].
Shiraishi Wataru,Une Hayato,Iwanaga Yasutaka,Yamamoto Akifumi
Rinsho shinkeigaku = Clinical neurology
A 36-year-old woman with a 4 year history of lower legs edema, hypermenorrhea and melena without medical treatment was admitted to our hospital. At 18 days before admission, anasarca and general fatigue appeared and she was admitted to another hospital. Her hemoglobin concentration was 1.4 g/dl and chest X-ray showed cardiomegaly. Heart failure with severe chronic anemia was diagnosed, and blood transfusion was performed. Her hemoglobin concentration increased to 10 g/dl and the anasarca disappeared. The day after discharge, she was referred to our hospital with generalized convulsion. We diagnosed posterior reversible encephalopathy syndrome (PRES) from the typical MRI imaging. We started treatment and her consciousness recovered steadily. At a week after admission, left hemiparesis appeared. Her brain imaging revealed multiple intracranial hemorrhages. In addition, her visual disturbance revealed vitamin A and vitamin K deficiency. PRES sometimes occur secondary to blood transfusion, but secondary brain hemorrhage is rare. Her fat-soluble vitamin deficiency, which resulted from a peculiar eating habit, may have contributed to the brain hemorrhage.
Posterior reversible encephalopathy syndrome in a postpartum woman with acute lymphoblastic leukaemia after intrathecal methotrexate.
Mescher Craig,Slungaard Arne
BMJ case reports
Posterior reversible encephalopathy syndrome (PRES) is the most common neurological complication occurring in children undergoing induction chemotherapy for acute lymphoblastic leukaemia (ALL) but is increasingly recognised to occur in adults as well. Here, we report a woman who presented with B-cell ALL (B-ALL) at the time of delivery and developed PRES 1 day after receiving intrathecal (IT) methotrexate (MTX) that rapidly resolved. She subsequently received IT MTX without recurrence of neurological symptoms. This case represents the first case of PRES in a postpartum B-ALL patient receiving IT MTX, demonstrates that re-treatment with MTX in this case could be done safely and highlights the risk of PRES in adults treated for B-ALL.
10.1136/bcr-2017-220429
Blood transfusion-related posterior reversible encephalopathy syndrome.
Zhao Zhen-Yu,He Feng,Gao Pei-Hong,Bi Jian-Zhong
Journal of the neurological sciences
Neurological complications have rarely been described after blood transfusion. Posterior reversible encephalopathy syndrome (PRES) is a recently recognized entity affecting predominantly the posterior cerebral hemispheres. We report two distinctive cases with history of chronic anemia that developed headache, blurred vision and seizure after blood transfusion. Magnetic resonance imaging indicated vasogenic edema consistent with PRES.
10.1016/j.jns.2014.05.001
Posterior Reversible Encephalopathy Syndrome and Reversible Cerebral Vasoconstriction Syndrome after Rapid Blood Transfusion.
Saito Kazuyuki,Shimizu Yu,Higuma Maya,Kubodera Takayuki,Wada Yoshiaki
Internal medicine (Tokyo, Japan)
We herein report a case of posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) that occurred immediately after blood transfusion. A 64-year-old Japanese woman was diagnosed with liver cirrhosis due to hepatitis B 2 years ago. She was admitted to our hospital with hemorrhagic shock due to esophageal variceal rupture. She was hospitalized with rapid blood pumping transfusion, after which consciousness disorder appeared, and her blood pressure suddenly increased. Magnetic resonance imaging revealed PRES and RCVS. We speculated that hypoalbuminemia and blood transfusion might have been involved in the development of PRES and RCVS.
10.2169/internalmedicine.1768-18
Reversible cerebral vasoconstriction syndrome following red blood cells transfusion: a case series of 7 patients.
Liang Hui,Xu Ziqi,Zheng Zhijun,Lou Haiyan,Yue Wei
Orphanet journal of rare diseases
BACKGROUND:Reversible cerebral vasoconstriction syndrome (RCVS) is an infrequent disease characterized by severe headaches with or without focal neurological deficits or seizures and a reversible vasoconstriction of cerebral arteries. The Orpha number for RCVS is ORPHA284388. However, RCVS triggered by blood transfusion is rare. Here we provided the clinical, neuroimaging and outcome data of patients diagnosed with RCVS resulting from red blood cells transfusion. METHODS:We retrospectively identified 7 patients presenting with RCVS after red blood cells transfusion from January 2010 to May 2014. The information on clinical features, neuroimaging and outcome were collected and analyzed. RESULTS:All 7 patients were Chinese women, with a mean age of 42 years (38-46). All the patients had severe anemia (Hb level < 6 g/dl) caused by primary menorrhagia due to uterine myoma (n = 5) or end-stage renal disease (n = 2) and severe anemia persisted for a average period of 4 months (2-6). Each patient received packed red blood cells transfusion (average: 1580 ml) over a period of 2-5 days. Blood transfusion increased the hemoglobin level by at least 4.5 g/dL from baseline. The neurological symptoms appeared a mean of 6.3 days (2-13) after the last blood transfusion. Headache was the most frequent symptom and seizure, transient or persistent neurological disorders were observed. Neuroimaging showed cortical subarachnoid hemorrhage (n = 2), focal intracerebral hemorrhage (n = 2), localized brain edema (n = 3), cerebral infarction (n = 1), and posterior reversible encephalopathy syndrome (n = 2). Cerebral vasoconstrictions were demonstrated by magnetic resonance angiography or cerebral angiography. Arterial constriction reversed in all patients within 1 to 3 months of follow-up after disease onset and no relapse was observed up to a mean of 17.1 ± 4.8 months of follow-up. CONCLUSIONS:RCVS is a rare complication as a result of blood transfusion in patients with chronic severe anemia and should be considered in patients who show severe headache or neurologic deficits after transfusion.
10.1186/s13023-015-0268-z
Posterior reversible encephalopathy syndrome following blood transfusion in a patient with factor X deficiency: Is it an unusual systemic manifestation of an adverse transfusion reaction?
Verma Anupam,Hemlata ,Elhence Priti,Phadke Shubha R,Neyaz Zafar
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis
Adverse neurological transfusion reactions including posterior reversible encephalopathy syndrome (PRES) following blood transfusion are rare. Our case an 18-year-female with known Factor X deficiency with menorrhagia developed severe hypertension, followed by generalised tonic clonic convulsions apparently after blood component transfusion. She had earlier received 4 units of red blood cells (RBC) for anaemia and 10 units of fresh frozen plasma (FFP) for menorrhagia (with prolonged PT and APTT) within short span of time at another hospital. There was no history of hypertension, convulsions, any cardiovascular, renal or neurological disease before transfusion. The clinical features and magnetic resonance imaging findings led to the diagnosis of PRES. Abnormal electroencephalogram and a hypercoagulable haemostatic profile on thromboelastography along with derangement in blood glucose and liver function tests were also observed. Patient responded well to the anticonvulsants and antihypertensive agents prescribed and was discharged in a stable condition. Our patient had a systemic transfusion reaction involving predominantly neurological system, however, cardiovascular, hepatic, haemostatic and endocrine systems were also affected. This case is unusual being the first report of PRES occurring in a patient with factor X deficiency presenting with an array of clinical and laboratory features which have not been reported in earlier studies involving PRES. Presumably the initial aggressive red cell transfusion to treat anaemia initiated the crisis and further large volumes of transfused FFP contributed to this adverse transfusion reaction in our case. Clinicians and Transfusion Medicine specialists should be aware about this uncommon clinical entity.
10.1016/j.transci.2017.11.030
Posterior reversible encephalopathy syndrome secondary to blood transfusion.
Singh Karanbir,Gupta Rajesh,Kamal Haris,Silvestri Nicholas J,Wolfe Gil I
Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
The appearance of posterior reversible encephalopathy syndrome (PRES) after blood transfusion is rare and has only been reported in three patients to our knowledge. We report a fourth patient with PRES secondary to blood transfusion. A 36-year-old woman with a history of menorrhagia presented to the emergency department with severe fatigue. She had a hemoglobin of 1.7 g/dl and received four units of red blood cells over 15 hours. On day 6 post-transfusion she returned with confusion, headache and a generalized tonic-clonic seizure. The MRI of her brain was consistent with PRES. The following day her confusion worsened, repeat MRI of the brain showed new T2-weighted lesions. Over next 10 days her mental status gradually improved close to her baseline. A repeat MRI of the brain showed resolution of the T2-weighted lesions. The clinical presentation, radiological findings and disease progression in our patient was consistent with PRES. Other than the blood transfusions, there were no apparent risk factors for PRES. The prior three patients with post-transfusion PRES have been reported in middle-aged women with uterine fibroids. It is suspected that these patients have a subacute to chronic anemic state due to ongoing menorrhagia. It is interesting to note that no cases of PRES post-transfusion have been reported in the setting of acute blood loss, such as from trauma. It is postulated that an abrupt increase in hemoglobin causes a rapid rise in blood viscosity and loss of hypoxic vasodilation. Subsequent endothelial damage and brain capillary leakage results in PRES. This constellation of changes may not occur after transfusion in patients with more acute blood loss.
10.1016/j.jocn.2014.10.005