Chronic kidney disease (CKD) is a well-known risk factor for venous thromboembolism and cardiovascular (CV) disease development in the general population, but its role in thrombotic risk in essential ...thrombocythemia (ET) and polycythemia vera (PV) remains poorly understood. This retrospective multicenter study analyzed clinical correlations and the potential impact of CKD on thrombosis development in ET and PV patients. We included 167 patients (76 ET and 91 PV); 25.7% had CKD at diagnosis, defined as estimated glomerular filtration rate (eGFR) < 60 mL/min/1.73 m
2
for ≥ 3 months. Lower eGFR correlated with advanced age, female sex, higher granulocytes, higher serum C-reactive protein, history of thrombosis, CV risk factors, and the presence of palpable splenomegaly. CKD was univariately associated with inferior thrombosis-free survival in the entire cohort, as well as in both ET and PV patients. These results remained significant in the multivariate Cox regression models when adjusted to disease-specific risk models. Therefore, CKD could be a risk factor for thrombosis in ET and PV patients. Additional studies on a larger number of patients are needed to confirm our findings and to elucidate whether the addition of CKD to the current risk stratification models might improve prognostication in ET and PV patients.
Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare autoimmune disorder characterized by auto-antibodies to Willebrand factor (vWF) cleaving enzyme (ADAMTS13), resulting in unusually large ...vWF multimers that lead to platelet aggregation, microthrombi formation and microangiopathic hemolytic anemia. Hemolysis in aTTP is mechanical; thus, direct antiglobulin test (Coombs test) is usually negative. Multiple autoimmune conditions and various auto-antibodies have been described in the context of chronic myelomonocytic leukemia (CMML). In this paper, we describe the first case of CMML with auto-antibodies to ADAMTS13, presenting initially as plasmapheresis-refractory Coombs-positive aTTP.
Although our patient was not treated for CMML, a complete remission of aTTP was eventually achieved with rituximab. Conclusion; We propose that aTTP should be in the differential diagnosis of CMML patients with thrombocytopenia and anemia (Coombs positive or not) who develop signs of thrombotic microangiopathy. Further studies are much needed to decipher the immune-mediated processes in CMML.
Despite being extremely rare, Guillain-Barre syndrome (GBS) has been recognized as a neurological complication of multiple myeloma, with variable responses to plasmapheresis (PEX), intravenous ...immunoglobulins (IVIG), and anti-myeloma therapies. In this paper, we report a case of a female patient with asymptomatic multiple myeloma (aMM) who initially presented as PEX- and IVIG-refractory GBS. After failure of PEX, IVIG, and anti-myeloma therapy (bortezomib, melphalan, and prednisone), the patient was eventually successfully treated with low-dose rituximab (100 mg/m.sup.2 per week in four doses). To the best of our knowledge, this is the first case to report successful treatment of refractory GBS potentially associated to aMM with low-dose rituximab. Additional studies are needed to elucidate the pathophysiological processes and the interplay between the dysregulated immune response, monoclonal immunoglobulin (MG), and neural tissue damage in GBS patients. Also, the potential role of rituximab in the treatment of MG-associated GBS warrants further exploration. Key words: Guillain-Barre syndrome; Rituximab; Monoclonal immunoglobulin; Multiple myeloma Guillain-Barreov sindrom (GBS) iznimno rijetko komplicira multipli mijelom. Nacin lijecenja ove neuroloske komplikacije u kontekstu multiplog mijeloma nije definiran s obzirom na to da plazmafereza, intravenski imunoglobulini i (imuno)kemoterapije usmjereni na plazmastanicni klon polucuju vrlo varijabilne klinicke odgovore. U ovom radu prikazujemo klinicki tijek bolesnice s asimptomatskim multiplim mijelomom (aMM) koja se pri dijagnozi prezentirala kao refraktorni GBS. Nakon neuspjesnog lijecenja plazmaferezom, imunoglobulinima i kemoterapijom (bortezomib, melfalan i prednizon) bolesnicu smo uspjesno lijecili niskim dozama rituksimaba (100 mg/m.sup.2 na tjedan u cetiri doze). Koliko nam je poznato, ovo je prvi slucaj u kojem je refraktorni GBS potencijalno povezan s aMM uspjesno lijecen niskim dozama rituksimaba. Dodatne studije su potrebne kako bi razjasnile patofizioloske procese izmedu monoklonskog imunoglobulina (MG) i ostecenja perifernog zivcanog sustava u bolesnika s GBS. Takoder, uloga rituksimaba u lijecenju bolesnika s GBS povezanim s MG zasluzuje dodatna istrazivanja. Kljucne rijeci: Guillain-Barreov sindrom; Rituksimab; Monoklonski imunoglobulin; Multipli mijelom
Despite being extremely rare, Guillain-Barré syndrome (GBS) has been recognized as a neurological complication of multiple myeloma, with variable responses to plasmapheresis (PEX), intravenous ...immunoglobulins (IVIG), and anti-myeloma therapies. In this paper, we report a case of a female patient with asymptomatic multiple myeloma (aMM) who initially presented as PEX- and IVIG-refractory GBS. After failure of PEX, IVIG, and anti-myeloma therapy (bortezomib, melphalan, and prednisone), the patient was eventually successfully treated with low-dose rituximab (100 mg/m
week in four doses). To the best of our knowledge, this is the first case to report successful treatment of refractory GBS potentially associated to aMM with low-dose rituximab. Additional studies are needed to elucidate the pathophysiological processes and the interplay between the dysregulated immune response, monoclonal immunoglobulin (MG), and neural tissue damage in GBS patients. Also, the potential role of rituximab in the treatment of MG-associated GBS warrants further exploration.
Author Affiliation: (1) Department of Internal Medicine, General Hospital of Sibenik-Knin County, Stjepana Radica 83, 22000, Sibenik, Croatia (2) Department of Internal Medicine, "Dr. Josip Bencevic" ...General Hospital, Slavonski Brod, Croatia (3) Department of Internal Medicine, General Hospital Zadar, Zadar, Croatia (4) Department of Psychiatry, General Hospital of Sibenik-Knin County, Sibenik, Croatia (5) University Hospital Dubrava, Zagreb, Croatia (6) School of Medicine, University of Zagreb, Zagreb, Croatia (a) krecak.ivan@gmail.com Article History: Registration Date: 01/14/2021 Received Date: 01/11/2021 Accepted Date: 01/14/2021 Online Date: 01/20/2021 Byline:
