hemolytic anemia treatment

Clostridium perfringens can rarely cause severe systemic infections, usually from an abdominal source, associated with massive hemolysis, which is usually fatal. Hemolytic anemia and acute renal injury resulting from toxin action are critical for the development of multiple organ dysfunction syndrome (MODs), making this condition a real emergency, requiring multispecialty skills and aggressive multimodal therapies. We herein describe a case of septic shock from acute cholecystitis with massive hemolysis caused by C...

An 11-year-old neutered male Jack Russell Terrier was presented to Yuki Animal Hospital for regenerative anemia during the treatment of hypoadrenocorticism. A blood smear examination showed spherocytes, polychromatic erythrocytes, and erythrocyte ghosts. The direct agglutination test was positive at 37°C. The dog was then diagnosed with immune-mediated hemolytic anemia (IMHA). Although prednisolone and mycophenolate mofetil were administered, the hematocrit and reticulocyte count decreased, and nonregenerative anemia developed...

Two Iranian patients with purine nucleoside phosphorylase (PNP) deficiency are described in terms of their clinical and molecular evaluations. PNP deficiency is a rare form of combined immunodeficiency with a profound cellular defect. Patients with PNP deficiency suffer from variable recurrent infections, hypouricemia, and neurological manifestations. Furthermore, patient 1 developed mild cortical atrophy, and patient 2 presented developmental delay, general muscular hypotonia, and food allergy. The two unrelated patients with developed autoimmune hemolytic anemia and T cells lymphopenia and eosinophilia were referred to Immunology, Asthma and Allergy Research Institute (IAARI) in 2019...

Gallstone (GS) disease is common and arises from a combination of genetic and environmental factors. Although genetic abnormalities specifically leading to cholesterol GSs are rare, there are clinically significant gene variants associated with cholesterol GSs. In contrast, most bilirubin GSs can be attributed to genetic defects. The pathogenesis of cholesterol and bilirubin GSs differs greatly. Cholesterol GSs are notably influenced by genetic variants within the ABC protein superfamily, including ABCG8, ABCG5, ABCB4, and ABCB11, as well as genes from the apolipoprotein family such as ApoB100 and ApoE (especially the E3/E3 and E3/E4 variants), and members of the MUC family...

Two severe cases of hemolytic anemia are described in different pediatric age groups, both linked to severe cobalamin deficiency from distinct causes. The first case refers to an exclusively breastfed infant with vitamin deficit secondary to maternal impaired absorption. Apart from the neurological deficits present at diagnosis, he also presented with infantile epileptic spasms syndrome a few months after treatment while having normal cobalamin serum levels. The second case refers to an adolescent with long-term inadequate intake...

BACKGROUND: Autoimmune hemolytic anemia (AIHA) is a rare, life-threatening disease in pediatrics. This article describes the clinical features, diagnostic workup, treatment and outcome in patients with AIHA. METHOD: Medical charts of under 18-year-old patients with AIHA treated at a tertiary Brazilian institution from 2006 to 2021 were retrospectively reviewed. Data analysis was primarily descriptive, using medians, interquartile ranges, and categorical variables presented as absolute frequencies...

Thrombotic microangiopathies (TMAs) comprise a distinct group of diseases with different manifestations that can occur in both pediatric and adult patients. They can be hereditary or acquired, with subtle onset or a rapidly progressive course, and they are particularly known for their morbidity and mortality. Pregnancy is a high-risk time for the development of several types of thrombotic microangiopathies. The three major syndromes are hemolysis, elevated liver function tests, and low platelets (HELLP); hemolytic uremic syndrome (HUS); and thrombotic thrombocytopenic purpura (TTP)...

Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare condition characterized by microangiopathic hemolytic anemia and kidney injury from thrombotic microangiopathy. P-aHUS occurs in approximately 1 in 25,000 pregnancies and is strongly related to complement dysregulation and pregnancy-related disorders, such as preeclampsia, eclampsia, and hemolysis, elevated liver enzymes, low platelet (HELLP) syndrome, resulting in adverse perinatal and fetal outcomes. Complement dysregulation in P-aHUS is commonly attributed to genetic mutations or autoantibodies affecting complement factors, including CFH , CFI , and MCP...

Babesiosis is a tick-borne parasitic infection seen in the Northeast and upper Midwest regions of the United States. Clinically, this intra-erythrocytic parasitic infection can present in a variety of ways, including fever, fatigue, malaise, or myalgia. Of note, these presenting symptoms are very similar to symptoms that can also be seen in patients with low-grade lymphoma. Thus, differentiating between babesiosis infection and active, symptomatic low-grade lymphoma can be difficult. We present a patient with concurrent severe babesiosis infection and follicular lymphoma...

Warm Autoimmune Hemolytic Anemia (WAHA) is the most common form of autoimmune hemolysis and there is a growing body of evidence of an association between SARS-CoV-2 infection, WAHA and a hyperinflammatory state, including hemophagocytic lymphohistiocytosis/macrophage activation syndrome. However, there is no literature to date of WAHA or hyperinflammatory state following administration of anti-SARS-CoV-2 monoclonal antibody treatment. This report documents a case of a patient with history of WAHA who developed brisk hemolysis and a hyperinflammatory state consistent with hemophagocytic lymphohistiocytosis/macrophage activation syndrome after COVID-19 infection and treatment with an anti-SARS-CoV-2 monoclonal antibody...

Autoimmune hemolytic anemia (AIHA) and hemophagocytic lymphohistiocytosis (HLH) are rare complications of infectious mononucleosis. The authors describe a 12-year-old male with acute infectious mononucleosis, hepatitis, cholestasis, and an autoimmune hemolytic disorder caused by cold agglutinins IgM (anti-i specificity). Clinical deterioration with persistent fever, anemia, and hepatosplenomegaly was consistent with cold AIHA plus concomitant HLH. The patient was treated with corticosteroids and acyclovir, with an uneventful recovery...

Zieve's syndrome is an underdiagnosed condition characterized by the triad of jaundice, hemolytic anemia, and hyperlipidemia in the setting of chronic alcohol use. It may be accompanied by acute alcoholic hepatitis. The distinction between the coexistence of acute alcoholic hepatitis with Zieve's syndrome and Zieve's syndrome in isolation is crucial, given the different treatments and prognoses in these situations. A 35-year-old woman presented with complaints of abdominal discomfort, nausea, and vomiting in the previous week...

BACKGROUND: Genetic underpinnings in Parkinson's disease (PD) and parkinsonian syndromes are challenging, and recent discoveries regarding their genetic pathways have led to potential gene-specific treatment trials. CASES: We report 3 X-linked levodopa (l-dopa)-responsive parkinsonism-epilepsy syndrome cases due to a hemizygous variant in the phosphoglycerate kinase 1 (PGK1) gene. The likely pathogenic variant NM_000291.4 (PGK1):c.950G > A;p.(Gly317Asp) was identified in a hemizygous state...

Dexamethasone (dex) is a potent glucocorticoid used to treat a variety of diseases. It is widely used in veterinary medicine in many species; for instance, in dogs, it can be used for emergent cases of anaphylaxis or trauma, management of immune-mediated hemolytic anemia or thrombocytopenia, certain cancers, allergic reactions, and topically for skin or eye inflammation. Dex is not without its side effects, especially when administered systemically, which might compromise compliance and effective treatment...

This case report describes an 18-month-old male presenting with hemolytic anemia and lethargy, who was ultimately diagnosed with severe vitamin B12 deficiency. The child exhibited global developmental delays, including a lack of speech and walking skills. Initially suspected as intravascular hemolytic anemia, the normal reticulocyte count led to further investigation, which revealed low cobalamin (vitamin B12) levels. The patient received vitamin B12 injections, resulting in normalized cobalamin levels. Additional evaluations ruled out metabolic disorders and other etiologies for his anemia...

OBJECTIVES: Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening disease of chronic uncontrolled complement pathway activation that leads to thrombotic-microangiopathy, along with severe organ damage, including end-stage kidney disease. This study aimed to evaluate the epidemiology, management, and outcome of aHUS in an Omani population. METHODS: This retrospective descriptive cohort study assessed all cases of aHUS diagnosed and followed up at two tertiary care centers in Oman from January 2008 to December 2019, based on clinical features, complement pathway assays, histopathological, and genetic testing...

Visceral leishmaniasis is a common tropical infection presenting with a myriad of hematological abnormalities. We report an unusual case of an 11-year-old girl suffering from a febrile illness with hepatosplenomegaly and anemia. Laboratory findings included pancytopenia and hyperbilirubinemia. The leishmania antigen rK39 was positive and bone marrow examination revealed hemophagocytosis and amastigote forms of Leishmania donovani. Direct Coombs' test was positive (warm type, IgG) and LDH was elevated. Tests for other infections were negative...

This retrospective cohort study described real-world treatment patterns and healthcare resource utilization (HCRU) of patients with warm autoimmune hemolytic anemia (wAIHA) initiating treatment with first-line (1L) oral corticosteroids (OCS) + rituximab (R) compared to 1L OCS. Patients with a wAIHA diagnosis code (D59.11) between 8/2020-3/2022 were identified using US pharmacy and medical claims databases. Patients initiating 1L OCS ± R were identified (date of initiation = 'index date') with a 1-year pre-index period and a variable (minimum 1-year) follow-up period...

Zieve's syndrome, associated with chronic alcoholism, manifests as hemolytic anemia, transient hyperlipidemia, and cholestatic jaundice. Key symptoms comprise nausea, abdominal pain, and jaundice. Diagnosis relies on recognizing the triad in those with an alcohol use history. Supportive management includes blood transfusions and alcohol cessation. The exact pathophysiology remains uncertain, with hypotheses ranging from alcohol-induced liver damage to autoimmune processes. The report emphasizes diagnostic complexities, particularly when concurrent with autoimmune disorders such as latent autoimmune diabetes of adults or complicated by disseminated intravascular coagulation (DIC)...

KEY CLINICAL MESSAGE: Aspirin-related hemolysis in G6PD deficiency could be late-onset during long-term administration. Hemolytic anemia could continue for a relatively long time in elder patient with G6PD deficiency, which might be related to other adverse events. ABSTRACT: Aspirin-related hemolysis in G6PD-deficient individuals was generally reported among patients who received high-dose supplements within several days after ingestion. The safety of long-term and low-dose (50-325 mg/day) aspirin in patients coexist G6PD deficiency and cardiovascular disease is neglected in clinical practice...