Negative Hemolytic Anemia Research Articles

An Unusual Case of Relapsing and Remitting Acute Kidney Injury

Paroxysmal nocturnal haemoglobinuria (PNH), although a rare type of acquired hemolytic anemia, can be life-threatening if not diagnosed early. Kidney involvement in PNH varies from reversible acute kidney injury to irreversible chronic damage. Here, we report a case of recurrent acute kidney injury in a young male requiring renal replacement support. Repeated history of AKI with coombs negative hemolytic anemia led us to perform PNH profile after ruling out other causes. Although kidney involvement in PNH is not apparent, this case shows the importance of having a high index of suspicion which will help in preventing further episodes of AKI and thus, chronic kidney disease burden.

Paroxysmal nocturnal haemoglobinuria presenting as cortical cerebral venous thrombosis

Abstract Paroxysmal nocturnal haemoglobinuria (PNH) arises as a consequence of mutation of phosphatidylinositol N-acetylglucosaminyltransferase subunit A gene that is required for synthesis of the glycosylphosphatidylinositol proteins to the cell surface of blood cells. Patients with PNH usually present with features of chronic unexplained anaemia, but can also present with bone marrow failure and thrombosis at unusual sites. We present the case of a 27-year-old who presented with complaints of headache and easy fatigability, on evaluation was found to have coombs negative haemolytic anaemia and brain magnetic resonance venogram revealed thrombosis in the left transverse and sigmoid sinuses. On further investigation, found to have PNH clone in both red and white blood cell lineages and diagnosed as PNH. Hence, any patient presenting with chronic non-immune intravascular haemolysis, unexplained anaemia and unusual thrombosis should alert the physician to consider PNH as a differential diagnosis to avoid delay in diagnosis and better management.

Overlapping features of thrombotic thrombocytopenic purpura and systemic lupus erythematosus, a diagnostic and a management challenge

Thrombotic thrombocytopenic purpura (TTP) is a rare, potentially fatal blood disorder that can be challenging for clinicians to identify in the setting of autoimmune diseases such as systemic lupus erythematosus (SLE). SLE can present with thrombotic microangiopathy (TMA) and differentiation between the two diseases can be quite difficult.
 In this case report we present a 62-year-old female with a history of SLE who was hospitalized with thrombocytopenia, dysarthria, dizziness, paresthesia and altered mental status. The laboratory results revealed direct Coombs negative hemolytic anemia, severe thrombocytopenia, significant elevation of lactate dehydrogenase, leukocytosis and presence of few schistocytes in the peripheral film. In addition, we also present evidence for circulating anti-ADAMTS13 antibody/ADAMTS13 antigen. Based on clinical, hematological, and biochemical findings, we concluded that it was a case of TTP-like microangiopathy associated with SLE and indicated treatment with methylprednisolone pulses and plasmapheresis. Later on, she developed psychosis and made plasmapheresis difficult to perform. We continued corticosteroids (dose 1mg/kg), RhoGAM, Chloroquine and Azathioprine, obtaining a satisfactory response after one week of treatment.

Direct antiglobulin test-negative autoimmune hemolytic anemia associated with erythropoiesis stimulating agent resistance in a CKD patient with IgG4-related disease: A case report

Rationale: Direct antiglobulin test (DAT)-negative autoimmune hemolytic anemia (AIHA) is an important differential diagnosis for erythropoiesis-stimulating agents-resistant renal anemia with hemolysis. Patient concerns: An 82-year-old female with a past medical history of diabetes, end-stage kidney disease (estimated glomerular filtration rate: 6.9 mL/min/1.73 m2), post-transcatheter aortic valve implantation, and IgG4-related retroperitoneal fibrosis on prednisolone therapy was referred to our department for assessment of severe anemia. The patient underwent immunohematological testing for serological evaluation. Diagnoses: DAT-negative AIHA and IgG4-RD was established based on DAT-negative and immunohematological tests. Interventions: Glucocorticoid was administered to the patient. Outcomes: The patient’s condition including hemoglobin level was relieved. Lessons: In chronic kidney disease, AIHA may contribute to the development of severe anemia and erythropoiesis-stimulating agents-resistance, irrespective of the results of Coombs test. Reticulocytosis, undetectable haptoglobin and a fall in hemoglobinA1c despite sustained hyperglycemia may be a clue to the diagnosis of hemolysis.

218 - COVID-19 and EBV co-infection leading to coombs negative hemolytic anemia and hepatitis in a healthy young adult

218 - COVID-19 and EBV co-infection leading to coombs negative hemolytic anemia and hepatitis in a healthy young adult

Atypical Hemolytic Uremic Syndrome Precipitated by Recurrent Pancreatitis

Atypical hemolytic uremic syndrome presents with negative direct antiglobulin test microangiopathic hemolytic anemia, acute kidney injury, and thrombocytopenia in the absence of diarrhea. Atypical hemolytic uremic syndrome may be triggered by drugs, infections, systemic lupus erythematosus, or, rarely, pancreatitis. Furthermore, recurrent hemolytic uremic syndrome should raise suspicion for complement-mediated atypical hemolytic uremic syndrome treated with eculizumab, an anti-C5 monoclonal antibody. Here, we report a case of complement-mediated atypical hemolytic uremic syndrome likely precipitated by recurrent alcohol-induced pancreatitis.

A Rare Case of Non-immune Hemolytic Anemia in a Stage IV Breast Cancer Patient Treated With Capecitabine

Incremental changes in the diagnosis of breast cancer leave drastic impacts on patients. There are detrimental shifts in cost, psychological disorders in terms of depression, and morbidities. Stage IV breast cancer has a high mortality rate and was afflicting our patient who was diagnosed with metastatic breast cancer estrogen receptor/progesterone receptor (ER/PR) positive, human epidermal growth factor receptor 2 (HER-2) neo negative, and low Ki-67. Among the various management modalities and effective treatments, capecitabine was selected because of its benefits; however, there are several commonly known adverse effects when using capecitabine including non-immune hemolytic anemia, a very rare and unexpected side effect despite the many research and clinical trials performed. Immune mediate or Coombs positive hemolytic anemia was reported with the usage of capecitabine, but this patient developed Coombs negative or non-immune hemolytic anemia. Capecitabine, a form of fluoropyrimidine, hypothetically affects the erythrocyte membrane structure resulting in the destruction of these cells. Additionally, discontinuation of capecitabine in the patient led to the resolution of the condition; this made us more aware of the precise diagnosis, also considering that the bone marrow biopsy came back negative.

WILSON’S DISEASE PRESENTING AS COOMB’S TEST NEGATIVE HEMOLYTIC ANEMIA

Wilson’s Disease (WD) is an autosomal recessive genetic disorder associated with excess storage of copper in liver, brain and other organs. Wilson ’s disease could be fatal if not diagnosed early and not treated. We report a 10year old female case presenting with discoloration of eyes, dark urine, generalized itching with loss of appetite and nausea turned out to be Wilson’s disease presenting as Coomb’s test negative Hemolytic Anemia and comorbid Rickettsial fever. Slit lamp examination revealed Kayser-Fleischer ring around the cornea, and 24-hour urinary copper and serum ceruloplasmin conrmed the diagnosis. Treatment aimed at preventing accumulation of copper in tissues and reducing already accumulated copper in the body. A combination of copper chelators like D-penicillamine and zinc therapy is used to prevent progress of WD. She was started on DPenicillamine with Zinc supplements and Tablet Doxycycline for Rickettsial fever. Prognosis and possibility of requirement of Liver transplantation at later stage of life explained to the attenders.

Rare case of self-limiting haemolysis associated with Dengue fever in a Sri Lankan female – case report

A 61-year old female with dengue haemorrhagic fever developed anaemia and rising transaminase levels on the 6th day of illness. She was found to have haemolysis with a negative direct antiglobulin test (DAT), and no red cell fragmentation. She recovered with supportive care. Haemolysis with associated Haemolytic uraemic syndrome (HUS), Disseminated intravascular coagulation (DIC) and cold Auto-immune haemolytic anaemia (AIHA) is reported previously; DAT negative haemolytic anaemia associated with dengue fever has not. This case suggests a need for further studies on other immune mechanisms that can lead to haemolysis with dengue fever.

Pregnancy-associated haemolytic anaemia: A cause not to be forgotten.

Anaemia in pregnancy is common, however, only a few cases of pregnancy-associated autoimmune haemolytic anaemia have been documented. Typically, such cases involve a positive direct antiglobulin test and have the potential to cause haemolytic disease of the fetus and newborn. Rarely, no autoantibodies are detected. We report two cases of direct antiglobulin test negative haemolytic anaemia occurring in multiparous women with no cause found. Both women had a haematological response to corticosteroid therapy and delivery.

Comparison of Polybrene DAT and Conventional DAT in Cases of Suspected DAT Negative Autoimmune Hemolytic Anemia (AIHA)

Abstract Introduction/Objective A negative Direct Antiglobulin Test (DAT) is seen in approximately 1-5% of patients presenting with signs and symptoms of immune hemolysis. A well-performed ‘standard’ DAT detects ~100-500 molecules of bound IgG per red cell. A more sensitive polybrene DAT may be helpful in order to confirm a diagnosis of AIHA in patients whose samples have tested negative via standard DAT. However, there has been little reported on utility of the polybrene DAT in evaluating such cases with modern DAT reagents. We hypothesized that the polybrene DAT would not contribute substantially to the analysis of Coombs-negative hemolytic anemia (C-NHA), based primarily on our anecdotal observation. As such, we undertook a study to assess results of polybrene DAT in cases evaluated for possible (C-NHA). Methods/Case Report Two study sites were used for data analysis (Yale-New Haven Hospital, Site A, and VA Connecticut, Site B) over a five year period (2016-2021). During this time, standard DATs were performed at both study sites by the tube method using polyspecific antiglobulin and, if positive, reflex to anti-IgG and -C3. For cases of suspected C-NHA (which are reported to Blood Bank via a consult mechanism), conventioned DAT- samples are referred to our regional immunohematology laboratory (American Red Cross, Farmington, CT) for manual hexadimethrine bromide (Polybrene) DAT. Polybrene DAT is reported as negative with two sources of polyspecific AHG. Subsets of patients also underwent acid elution studies (Gamma ELU-KITII) as part of a C-NHA algorithm developed during the study period. Results of standard DAT, acId elution, and polybrene DAT were extracted from Blood Bank electronic records at both study sites. Results (if a Case Study enter NA) Evaluation for C-NHA was performed in 32 patients/cases over the study period. Amongst these individuals, 96.8% (31/32) underwent polybrene DAT assessment and none (0%; 0/31) demonstrated a positive polybrene DAT result. Notably, acid elution studies were performed in 90.6% (29/32) of traditional DAT negative cases. Of these, 10.3% (3/29) had reactive eluates. Conclusion Performance of the polybrene DAT appeared to be of no value in the assessment of suspected C-NHA. Given that nearly 10% of individuals with a negative conventional DAT had a positive acid elution, this testing step appears to be of greater value in potentially identifying an autoantibody in suspected cases of C-NHA.

C3 glomerulopathy and atypical hemolytic uremic syndrome: an updated review of the literature on alternative complement pathway disorders.

The complement system plays a significant role within the pathological process of C3 glomerulopathy (C3GP) and atypical hemolytic uremic syndrome (aHUS). In daily practice, clinicians should differentiate the subgroups of C3GP because of they should apply different treatment modalities. In the past, C3GP was considered as a part of membranoproliferative glomerulonephritis (MPGN). MPGN is defined as glomerular capillary thickening secondary to the synthesis of the new glomerular basement membrane and mesangial cellular hyperplasia with mesangial matrix expansion. Atypical hemolytic uremic syndrome is an ultra-rare disease that can be outlined by the triad of Coombs negative microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Recent advances demonstrated that these diseases share common abnormalities of the control of the alternative complement system. Therefore, nowadays, most researchers advocate that there may be overlap in the pathogenesis of C3GP and aHUS. This review will provide recent novel mechanisms and treatment options in these diseases. For the purposes that we mentioned above and to help clinicians, we aimed to describe the etiology, pathophysiology, and treatment of C3GP and aHUS in this comprehensive review.

Wilson Disease-Induced Acute Liver Failure (NWI = 13) Salvaged without Liver Transplant by Plasmapheresis

AbstractWilson disease (WD) is a disorder of copper metabolism resulting in accumulation of copper in vital organs of the human body, predominantly in the liver and the brain. Acute liver failure in WD has a bad prognosis, especially with a score ≥11 in the revised WD prognostic index; emergency liver transplantation is considered the only life-saving option in this scenario. Here, we reported a girl patient with WD-induced liver failure and poor prognostic score who was rescued by plasmapheresis. She also manifested severe Coombs negative hemolytic anemia and acute kidney injury. This case report highlights the utility of an adjunctive modality besides liver transplantation for the management of fulminant liver failure caused by WD.

Heme oxygenase-1 deficiency presenting with interstitial lung disease and hemophagocytic flares

BackgroundHeme oxygenase-1 (HMOX1) catalyzes the metabolism of heme into carbon monoxide, ferrous iron, and biliverdin. Through biliverdin reductase, biliverdin becomes bilirubin. HMOX1-deficiency is a rare autosomal recessive disorder with hallmark features of direct antibody negative hemolytic anemia with normal bilirubin, hyperinflammation and features similar to macrophage activation syndrome. Clinical findings have included asplenia, nephritis, hepatitis, and vasculitis. Pulmonary features and evaluation of the immune response have been limited.Case presentationWe present a young boy who presented with chronic respiratory failure due to nonspecific interstitial pneumonia following a chronic history of infection-triggered recurrent hyperinflammatory flares. Episodes included hemolysis without hyperbilirubinemia, immunodeficiency, hepatomegaly with mild transaminitis, asplenia, leukocytosis, thrombocytosis, joint pain and features of macrophage activation with negative autoimmune serologies. Lung biopsy revealed cholesterol granulomas. He was found post-mortem by whole exome sequencing to have a compound heterozygous paternal frame shift a paternal frame shift HMOX1(NM_002133.3):c.262_268delGCCCTGGinsCC (p.Ala88Profs*51) and maternal splice donor HMOX1 (c.636 + 2 T > A) consistent with HMOX1 deficiency. Western blot analysis confirmed lack of HMOX1 protein upon oxidant stimulation of the patient cells.ConclusionsHere, we describe a phenotype expansion for HMOX1-deficiency to include not only asplenia and hepatomegaly, but also interstitial lung disease with cholesterol granulomas and inflammatory flares with hemophagocytosis present in the bone marrow.

PULMONARY MUCORMYCOSIS: A RARE CAUSE OF HYDROPNEUMOTHORAX

SESSION TITLE: Medical Student/Resident Chest Infections Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Mucormycosis is a rare but often fatal fungal infection. These fungi are ubiquitous in nature and most commonly cause rhino-orbital-cerebral infections. Patients who are immunocompromised are at higher risk (1). CASE PRESENTATION: A 76 year-old never-smoker with a medical history significant for type 2 diabetes (HbA1c 5.8%) and Coombs negative hemolytic anemia was admitted with 3 months of progressive dyspnea. She had an associated nonproductive cough for 10 days. She denied fevers, night sweats, chest pain or hemoptysis. Two months prior to presentation, she started prednisone 60 mg daily for hemolytic anemia. Prednisone was slowly tapered to 30 mg daily at the time of presentation. She received one dose of Rituximab due to persistent anemia. Evaluation 2 weeks prior to presentation revealed a right upper lobe, 5 cm cavitary lesion on chest CT. On arrival she was tachycardic (heart rate 110) and tachypneic (respiratory rate 32) without hypoxemia. Labs were notable for a hemoglobin of 9.5 g/dl, WBC of 13.6, and creatinine of 1.85 mg/dl. Chest x-ray showed a loculated right hydropneumothorax. Chest CT showed the right hydropneumothorax with progression of the right upper lobe cavitary lesion. A 14 French pigtail catheter was placed at bedside via ultrasound guidance. Pleural studies demonstrated exudative fluid with negative cytology and cultures. She underwent CT-guided biopsy of the cavitary lesion, which revealed inflammation, necrosis, and numerous broad-ribbon-like, pauci-septate hyphae favoring mucormycosis. IV liposomal amphotericin and posaconazole were initiated. Thoracic surgery declined operative intervention due to her age and comorbidities. She developed respiratory and renal failure and passed away 1 month after presentation. DISCUSSION: This patient developed pulmonary mucormycosis in the setting of glucocorticoid and Rituximab therapy. The diagnosis of mucormycosis can be difficult due to nonspecific symptoms (1) and lack of serologic tests. Commonly used fungal antigen tests such as 1,3-beta-D-glucan are traditionally negative (2). Chest CT findings are nonspecific with the classic halo or reversed halo sign present in only a minority of cases (1). A definitive diagnosis is made with histopathology (1). Successful treatment requires early diagnosis, reversal of predisposing factors, surgical debridement, and prompt initiation of antifungal therapy (3). Liposomal amphotericin is first-line pharmacologic therapy (1); however, antifungal therapy without surgical debridement is unlikely to be successful. CONCLUSIONS: Our patient presented with an unusual case of pulmonary mucormycosis; a high suspicion and histopathology are required to make the diagnosis. CT findings are non-specific and common fungal antigen tests can be negative. Early diagnosis and prompt initiation of antifungal therapy with surgical debridement improves the chance for survival. Reference #1: Farmakiotis D, Kontoyiannis DP. Mucormycoses. Infect Dis Clin North Am 2016; 30:143. Reference #2: Ostrosky-Zeichner L, Alexander BD, Kett DH, et al. Multicenter clinical evaluation of the (1-->3) beta-D-glucan assay as an aid to diagnosis of fungal infections in humans. Clin Infect Dis 2005; 41:654. Reference #3: Spellberg B, Walsh TJ, Kontoyiannis DP, et al. Recent advances in the management of mucormycosis: from bench to bedside. Clin Infect Dis 2009; 48:1743. DISCLOSURES: No relevant relationships by Meghan Hill, source=Web Response author/editior relationship with UpToDate Please note: $5001 - $20000 Added 04/01/2020 by David Midthun, source=Web Response, value=Royalty No relevant relationships by Kelly Pennington, source=Web Response

Haemolytic Anaemia: A Diagnostic Dilemma

It is sometime difficult to find out the cause of haemolysis in haemolytic anaemia due to inconclusive results of investigation. Diagnosis of immune haemolytic anaemia is often difficult when Coomb’s test is negative. Here we present such a case of fourteen years old girl who presented with haemolytic anaemia with negative Coomb’s test. Due to high clinical suspicion we labelled her as a case of Coomb’s negative immune haemolytic anaemia and she responded well to steroid.

Anemia hemolítica como presentación inicial de la enfermedad de Wilson: A propósito de una enfermedad rara con una presentación infrecuente

La enfermedad de Wilson es un raro trastorno genético que afecta la capacidad de excreción del cobre. Su distribución es mundial, con una prevalencia estimada en 30 casos por millón de habitantes. Aunque los síntomas más frecuentes son los de origen hepático y neuropsiquiatricos, la anemia hemolítica con Coombs negativo puede ser la única manifestación de la enfermedad y su presentación suele preceder por meses a la enfermedad hepática clínicamente evidente o las manifestaciones neurológicas. Se presenta el caso de una paciente joven con anemia hemolítica Coombs negativo y relación fosfatasa alcalina/bilirrubina total <4 y AST/ALT >2,2, en quien termino por establecerse como diagnostico una enfermedad de Wilson.

Comparison of αLEPRA and αLELY Co-Inheritance with a SPTA1 Whole Gene Deletion, a Family Study

Background: Genetic variants resulting in quantitative defects in the production of α-spectrin are associated with autosomal recessive hereditary spherocytosis (HS). Because erythroid cells produce α-spectrin in a 3-4:1 ratio in relation to β-spectrin, one defective allele is typically well-tolerated and a single null allele combined with a second partially defective allele may display a sub-clinical phenotype. Protein expression can be reduced by nonsense, frameshift, missense or splicing variants but reported large deletion events are extraordinarily rare (n = 1). Splicing variants include the low expression alleles αLELY - Low Expression LYon (SPTA1:c.6531-12C>T, p.?) and αLEPRA-Low Expression PRAgue (SPTA1:c.4339-99C>T, p.?) which reduce protein expression by 50% and 16%, respectively. Here, we present a family study with a whole gene SPTA1 deletion that characterizes the patient phenotype when a full gene mutation is co-inherited with the αLEPRA and αLELY low expression alleles. Methods: The 6 month-old male proband had a history of congenital Coombs negative hemolytic anemia requiring transfusion from 3 months of age and in whom a red cell membrane disorder was clinically suspected. Pretransfusion CBC and peripheral blood smear results were unknown. Transfused CBC indices were as follows: RBC 4 x 1012/L, HGB 11 g/dL, MCV 76 fL, MCH 27 pg, MCHC 36 g/dL and a reticulocyte count of 1.8%. The proband's mother, of Cuban descent, and father, of European descent, had no history of anemia or HS. Targeted high throughput sequencing of genes related to hereditary hemolytic anemia was performed for the detection of single nucleotide variants (SNVs) and small insertion/deletion variants (indels). Relative quantitative real time PCR (RT-PCR) was utilized to confirm large structural variants. Results: Sequencing identified a large heterozygous deletion that spanned the entire SPTA1 gene (NC_000001.11:g.(?_158656337)_(158581024_?) in the proband and his mother. In addition, the proband had inherited hemizygous αLEPRA from his father. The mother had hemizgous αLELY. The father showed heterozygous αLEPRA and no other SPTA1 variants (Table 1). Conclusion: To our knowledge, only Iolascon et al in 2011 has reported a SPTA1 gene large deletion (SpectrinExeter, exon 11-exon 12 deletion, loss of 83 amino acids) which resulted in a structurally abnormal protein and ellipto-poikilocytosis phenotype when in trans to αLELY. Our current case is the first reported SPTA1 full gene deletion and our family study allows comparison of the effects of coinheritance of the low expression alleles αLEPRA and αLELY. When in combination with complete absence of the SPTA1 gene in trans configuration, αLELY manifests in subclinical effects and αLEPRA causes a recessive HS phenotype, which resulted in severe anemia. This contrast further demonstrates that, unlike αLEPRA, αLELY does not decrease the α-spectrin concentration sufficiently to contribute to the baseline phenotype regardless of the severity of the second allele mutation for quantitative disorders such as HS. Disclosures No relevant conflicts of interest to declare.

SUN-038 RECOVERY OF KIDNEY FUNCTION IN GLOMERULONEPHRITIS WITH RAPIDLY PROGRESSIVE RENAL FAILURE AT CHO RAY HOSPITAL

It is uncommon for patients with glomerulonephritis (GN) who require hemodialysis (HD) to recover renal function. Lupus nephritis (LN) is one of the common GN in Vietnam. Our aim was to describe the renal outcome of 64 GN patients with rapidly progressive renal failure (RPRF) on HD at Cho Ray Hospital, Ho Chi Minh City, Vietnam. A prospective study was conducted in 64 GN patients with RPRF requiring HD from 2014 to 2018. RPRF was defined as a progression to renal failure within a period of 1 week to three months. A definitive diagnosis of the kidney disease was established by history, clinical examination, lab tests, and renal biopsy. The patients had received standard therapy for their primary medical condition. We followed the patients after initiation of HD (3 months min, 18 months max) to evaluate recovery sufficient to discontinue HD Patient median age 25 (Inter-quartile range 21,32); 49 females; 59/64 pts with lupus nephritis (LN) (54 class III or IV, 1 class V, and 1 unclassified). 3 patients had IgA nephropathy; 1 Anti-glomerular basement membrane GN; 1 idiopathic membranoproliferative GN. Thrombotic microangiopathy (TMA) was clinically diagnosed based on Coombs negative hemolytic anemia, thrombocytopenia, D-Dimer >600 ng/mL, LDH >600 U/L, schistocytes on blood smear, with or without kidney biopsy. TMA was found in 56 pts, of which 19 were biopsy proven. Six LN patients with TMA were tested for ADAMTS13, all of whom were negative. Patients with TMA were treated with therapeutic plasma exchange (26 cases) or plasma infusion (30 cases) and heparin anticoagulation. All 64 pts were on oral methylprednisolone (median 32mg/day, IQR 16, 48mg) for the first month then tapered. 41 pts (39 LN, 2 non Lupus GN) received median of 6 doses of monthly intravenous cyclophosphamide 0.5g/m2 over median 12 months follow up. 36/59 (61%) of LN pts recovered kidney function, and discontinued HD after median 39 days (IQR 24;72 days). They remained dialysis independent for a median of 10 months (IQR 6 ,11 months) follow up. Median of the last serum creatinine in patients who recovered function was 1.4 mg/dL (IQR 1.2; 1.7 mg/dL). In the 28 patients who failed to recover kidney function, 4 died and 24 continued HD after median 4 months. Recovery of renal function was more common in LN pts with TMA or patients with length on HD of less than 3. Recovery of function was not associated with age, gender, body mass index, hypertension, 24h proteinuria, hematuria, isolated TMA diagnosis, anemia, or serum albumin on admission). TableComparison between patients who recovered and failed to recover functionTotal (N=64)Recovered kidney function (N=36)Non recovered kidney function (N=28)pLupus nephritis5936 (61%)230.03Nonlupus GN50 (0%)5TMAneg/LNpos85 (62.5%)30.01TMApos/LNpos5131(60.7%)20TMApos/LNneg50(0%)5Length on HD ≤ 3 months4632(69.5%)140.005Length on HD > 3 months184(22.2%)14 Open table in a new tab RPRF in lupus nephritis patients with or without TMA had a good likelihood of function recovery after requiring hemodialysis.

A Multimodality Approach to Assessing Factor I Genetic Variants in Atypical Hemolytic Uremic Syndrome

A Multimodality Approach to Assessing Factor I Genetic Variants in Atypical Hemolytic Uremic Syndrome