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Influenza A H1N1 Triggering Complement-Mediated Hemolytic Uremic Syndrome.

Complement-mediated hemolytic uremic syndrome (CM-HUS) is a condition characterized by hemolytic anemia, thrombocytopenia, and renal impairment, typically associated with a chronic dysregulation of the complement system. Few adult cases of CM-HUS triggered by influenza infection have been described. Here we present a case of a 56-year-old female with multiple myeloma receiving cancer treatment who presented with Influenza A H1N1-associated CM-HUS. She was treated with anti-complement therapy and at her 1-month follow-up had complete resolution of renal dysfunction and hemolytic anemia. This ca

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… test. … Typical cases of iHUS present with acute kidney injury with proteinuria; MAHA with elevated LDH, presence of schistocytes, thrombocytopenia, negative Coombs testing; evidence of fibrinolysis with elevated D-dimer and low fibrinogen levels; normal to low C3 and C4 complement levels; and often a genetic …

Artificial intelligence for risk assessment and outcome prediction in malignant haematology.

Risk stratification is the backbone of clinical decision-making for patients with haematological malignancies. Models currently used in routine clinical practice often rely on static rules, conventional statistics and expert consensus. While pragmatic and accessible, these models fail to capture the complex biological interactions and patient heterogeneity, especially as data dimensionality grows given the availability of more comprehensive molecular analysis. Artificial intelligence (AI), particularly machine learning (ML), offers the potential to process such high-dimensional data, uncover l

Case Report: First occurrence of smoldering multiple myeloma in activated phosphoinositide 3-Kinase δ syndrome.

Activated phosphoinositide 3-kinase δ syndrome (APDS) is a newly described inborn error of immunity. Heterozygous mutations in the PI3Kδ catalytic subunit p110δ (PIK3CD) or regulatory subunit p85α (PIK3R1) lead to APDS1 and APDS2, respectively. Clinical manifestations include lymphoproliferation, recurrent respiratory tract infections, herpesvirus infections, enteropathy, elevated IgM, autoimmunity, and increased susceptibility to tumors, particularly lymphoma. Smoldering multiple myeloma (SMM), an asymptomatic clonal plasma cell disorder considered a pre-malignant entity, may progress to symp

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… The direct Coombs test was positive (+ +), while the indirect Coombs test was negative. Biochemical tests indicated an albumin level of 37.9 g/L (NR, 40–55 g/L), with normal globulin, liver enzymes (ALT, AST), bilirubin, and renal function. …

Missed Until Critical: Unravelling the Mystery of Persistent Hemolysis With a Definitive Diagnosis of Babesiosis.

Babesiosis is a parasitic protozoan infection caused primarily by Babesia microti in the United States. Its presentation can imitate that of malaria, which often leads to misdiagnosis. We present here a case of a 54-year-old female with multiple comorbidities, such as hypertension and hyperlipidemia, who was initially diagnosed with malaria but, with further investigation, was found to have babesiosis. The patient initially presented with anemia and thrombocytopenia, prompting an extensive and meticulous workup including a peripheral blood smear and autoimmune studies. From these tests, we wer

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… However, the lack of a positive Coombs test and the eventual identification of intraerythrocytic parasites pointed toward an infectious etiology, steering the clinical team away from corticosteroids or other immunosuppressive agents, which could have worsened the underlying parasitic infection. …

Hematology

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… Test direct coombs test (DAT)—patient’s washed RBC incubated with anti-IgG and anti-C3. … Anemia, smear (spherocytosis), ↑ reticulocytes, ↑ bilirubin, ↑ LDH, ↓ haptoglobin, direct Coombs test (IgG–, C3+), cold agglutinin screen treatments — symptom control (avoidance of cold). Chemotherapy (cyclophosphamide, chlorambucil). Biological agents (rituximab, INFα). …

Primary and Secondary Immunodeficiency Diseases in Oncohaematology: Warning Signs, Diagnosis, and Management.

Background: Immunodeficiencies (ID), in particular primary immunodeficiencies (PID), are often associated with haematological manifestations, such as peripheral cytopenias or lymphoproliferative syndromes. Early diagnosis and management have significant prognostic implications. Secondary immunodeficiencies (SID) may also be induced by oncohaematological diseases and their treatments. Haematologists and oncologists must therefore be aware of the association between blood disorders and cancer and ID, and be prepared to offer their patients appropriate treatment without delay. Our aim was to defi

EMA Review of Daratumumab for the Treatment of Adult Patients with Multiple Myeloma.

On May 20, 2016, a conditional marketing authorization valid through the European Union (EU) was issued for daratumumab as monotherapy for the treatment of adult patients with relapsed and refractory multiple myeloma, whose prior therapy included a proteasome inhibitor (PI) and an immunomodulatory drug (IMiD) and who had demonstrated disease progression on the last therapy. The review of daratumumab was conducted under the EMA's accelerated assessment program for drugs that are of major interest for public health, especially from the point of view of therapeutic innovation.Daratumumab monother

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… Interference with blood typing was another important identified risk reported with daratumumab because binding to CD38, found also at low levels on red blood cells (RBCs), may result in a positive indirect Coombs test. … A daratumumab‐mediated positive indirect Coombs test may persist for up to 6 months after the last daratumumab infusion. It should be recognized that daratumumab bound to RBCs may mask detection of antibodies to minor antigens in the patient's serum. …

Прогресс в патофизиологии и лечении анемии в множестве миеломы.

ЦЕЛЬ РАССМОТРЕНИЯ: Анемия является общим признаком во многих миеломах благодаря многофакторным механизмам. Помимо хорошо известных факторов, связанных с этим заболеванием, в последнее время использование ряда новых лекарственных средств в качестве иммунотерапевтических указывает на формирующуюся роль связанных с наркотиками механизмов в патофизиологии анемии у многих пациентов, страдающих миеломой. ПОСЛЕДНИЕ ФИННДИНЫ: Анемия, связанная с иммуномодулятивными препаратами, может быть вызвана как непосредственным воздействием на гематопоиетические клетки потомства, так и косвенным воздействием one

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… Several studies have shown that despite persistent Coombs test positivity, pts receiving daratumumab rarely exhibit biochemical or haematological markers of haemolysis [ 33 ]. … Consequently, a positive Coombs test in this clinical context must be interpreted with caution and always correlated with clinical findings and laboratory evidence of haemolysis. …

Mutations in the alternative complement pathway in multiple myeloma patients with carfilzomib-induced thrombotic microangiopathy.

Thrombotic microangiopathy (TMA) has been reported to occur in multiple myeloma (MM) patients in association with treatment with carfilzomib, an irreversible proteasome inhibitor (PI). The hallmark of TMA is vascular endothelial damage leading to microangiopathic hemolytic anemia, platelet consumption, fibrin deposition and small-vessel thrombosis with resultant tissue ischemia. The molecular mechanisms underlying carfilzomib-associated TMA are not known. Germline mutations in the complement alternative pathway have been recently shown to portend increased risk for the development of atypical

Prevalence and associated factors of medication non-adherence in hematological-oncological patients in their home situation.

BackgroundMedication non-adherence is associated with poor health outcomes and increased health care costs. Depending on definitions, reported non-adherence rates in cancer patients ranges between 16 and 100%, which illustrates a serious problem. In malignancy, non-adherence reduces chances of achievement of treatment response and may thereby lead to progression or even relapse. Except for Chronic Myeloid Leukemia (CML), the extent of non-adherence has not been investigated in hematological-oncological patients in an outpatient setting. In order to explore ways to optimize cancer treatment res

Concomitant primary hyperparathyroidism and systemic lupus erythematosus: coincidence or not? A new case report.

Primary hyperparathyroidism (PHP) is the most common cause of hypercalcemia. Patients with systemic lupus erythematosus (SLE) can develop hypercalcemia but it is exceptionally due to PHP. There are only few cases of concurrent SLE and primary hyperparathyroidism (PHP) described in the literature. We report a case of a 31-year-old patient having SLE with lupus nephritis class III and anti-phospholipid syndrome, complicated by pulmonary embolism associated to primary hyperparathyroidism causing severe hypercalcemia and osteoporosis. Even if there is no evidence for potential pathogenic associati

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… Direct coombs test was negative. Serum Albumin was 25g/l; proteins level was low at 50g/l and proteinuria at 3.2g/day. Creatinine was normal. Anti-nuclear antibody ANA were positive 1/320 with homogeneous pattern ( Figure 1 ), anti-dsDNA level was 160U/ml. …

Best Practice for the Administration of Daratumumab in Multiple Myeloma: Australian Myeloma Nurse Expert Opinion.

Patients with multiple myeloma (MM) are typically of an advanced age and may have significant co-existing medical conditions. They have often had multiple lines of therapy and as such experience disease-related effects alongside associated treatment toxicities. Daratumumab is a monoclonal antibody approved for the treatment of MM in the relapsed/refractory setting. Clinical studies found that daratumumab showed good tolerability as a monotherapy and in combination with current standard therapies. However, the administration of daratumumab does require specific management considerations. It is

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… Daratumumab does not interfere with ABO/RhD typing, but does give false-positive results for the indirect antiglobulin test (IAT; Coombs test), which is used for the detection of irregular blood group antibodies.[ 27 , 28 ] As a result, it is important that blood typing is done prior to administration … Blood typing Daratumumab gives false-positive results for the IAT; Coombs test. Patients should obtain a baseline red blood cell phenotype 1–3 days prior to starting daratumumab therapy so that blood provided for transfusion is of the correct phenotype. …

A Comprehensive Review of the Diagnosis and Management of Acute Liver Failure.

Acute liver failure (ALF) is a rare and specific form of severe hepatic dysfunction characterized by coagulopathy and hepatic encephalopathy in a patient with no known liver disease. ALF carries a high morbidity and mortality. Careful attention should be given to hemodynamics and metabolic parameters along with the active surveillance of infections. Timely transfer and supportive management are important in an intensive care unit in a liver transplant center. Identifying patients who will and will not improve with medical management and may need emergent liver transplantation is critical. In t

Allergy in Cancer Care: Antineoplastic Therapy-Induced Hypersensitivity Reactions.

As the backbone of oncological treatments, systemic chemotherapy is still one of the main pawns in cancer care, alone or in combination with newer targeted agents. All chemotherapy agents can be associated with a type of adverse event called an infusion reaction, which can be characterized as unpredictable, non-dose related, and unexplained by the cytotoxic profile of the drug. For some of these events, a certain immunological mechanism can be identified by blood or skin testing. In this case, we can speak of true hypersensitivity reactions that occur as a response to an antigen/allergen. The

Carfilzomib Induced Microangiopathy due to Accumulation With Paxlovid.

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… The diagnosis of hemolytic microangiopathic anemia was established on the basis of the following results: hemoglobin 9.3 g/dl, lactate dehydrogenase 716 U/l (normal < 246), haptoglobin < 0.08 g/l, with schistocytes on peripheral blood smear, with a negative direct Coombs test. …

Thrombotic microangiopathy during carfilzomib use: case series in Singapore.

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Major approaches in early diagnostics of common variable immunodeficiency in adults in Moscow.

Common variable immunodeficiency (CVID) is a primary immunological disease characterized predominantly by hypogammaglobulinemia. The main clinical manifestations are severe recurrent infections that often lead to structural damage of affected organs. The early start of adequate intravenous immunoglobulin therapy has significantly improved the prognosis of this serious disorder. Patients with CVID are also predisposed to autoimmune and lymphoproliferative complications. This article deals with the features of this primary immunodeficiency in adults. Clinical manifestations, immunological featur

Novel B55α-PP2A mutations in AML promote AKT T308 phosphorylation and sensitivity to AKT inhibitor-induced growth arrest.

Activation of the Protein Kinase B (PKB), or AKT pathway has been shown to correlate with acute myeloid leukemia (AML) prognosis. B55α-Protein Phosphatase 2A (PP2A) has been shown to dephosphorylate AKT at Thr-308 rendering it inactive. In fact, low expression of the PP2A regulatory subunit B55α was associated with activated phospho-AKT and correlated with inferior outcomes in AML. Despite this fact, no studies have specifically demonstrated a mechanism whereby B55α expression is regulated in AML. In this study, we demonstrate novel loss of function mutations in the PPP2R2A gene identified in

Successful treatment of an adult with Kasabach-Merritt syndrome using thalidomide, vincristine, and prednisone.

ObjectiveKasabach-Merritt syndrome is a rare disease that mainly occurs in infants and adolescents. It usually manifests as disseminated intravascular coagulation and severe bleeding, and is associated with high mortality. However, its low incidence and clinical rarity in adults mean that there is currently no well-verified treatment regimen for this disease. We report on an effective novel therapeutic regimen in a patient with Kasabach-Merritt syndrome.MethodsA woman with Kasabach-Merritt syndrome presented with a recurrent subcutaneous mass and disseminated intravascular coagulation, and was

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… Hemolytic studies, including Coombs test, glucose 6-phosphate dehydrogenase activity, erythrocyte permeability experiments, and CD55/CD59 were all normal. Serum protein electrophoresis and immunofixation showed no monoclonal band. Cytomegalovirus and Epstein-Barr virus DNA tests were both negative. …

Rationale and design of the German-speaking myeloma multicenter group (GMMG) trial HD6: a randomized phase III trial on the effect of elotuzumab in VRD induction/consolidation and lenalidomide maintenance in patients with newly diagnosed myeloma.

BACKGROUND: Despite major advances in therapy, multiple myeloma is still an incurable malignancy in the majority of patients. To increase survival, deeper remissions (i.e. CR) translating into longer PFS need to be achieved. Incorporation of new drugs (i.e. bortezomib and lenalidomide) as induction and maintenance treatment in an intensified treatment concept, including high dose melphalan (200 mg/m2), has resulted in increased CR rates, and is considered the standard of care for younger patients. Elotuzumab in combination with lenalidomide and dexamethasone has given better results as lenalid