warfarin and Anemia--Sickle-Cell

Here we describe an 8-year old male child with homozygous sickle cell disease who presented with left parietal skull bone infarction and, during his stay in hospital, developed a right femoral deep vein thrombosis (DVT), both uncommon complications of the disease. He initially presented with severe headache and generalised tenderness of the calvarium, which did not respond to simple analgesics. Scalp swelling in and around the left frontal (including left orbit) and parietal regions developed 24 h after presentation. The differential diagnosis included incipient stroke, acute sickle bone crisis and osteomyelitis, with a possible complication of epidural haematoma, or orbital compression syndrome. An initial exchange blood transfusion did not lead to appreciable reduction in opiate requirements. Significant symptomatic relief was attained only after a second exchange transfusion. The DVT developed at the site of catheterisation (right femoral vein), and this was treated with maximal doses of enoxaparin followed by warfarin. The child is now well and off anti-coagulants. In this article we present a review of the literature and discuss possible mechanisms of these complications in our patient.

Topics: Anemia, Sickle Cell; Anticoagulants; Catheterization; Child; Diagnosis, Differential; Edema; Enoxaparin; Exchange Transfusion, Whole Blood; Femoral Vein; Headache; Humans; Infarction; Male; Osteomyelitis; Parietal Bone; Stroke; Thrombophilia; Thrombophlebitis; Warfarin

To evaluate the activation of clotting systems in patients with sickle cell disease (SCD) by measuring the plasma D-dimer level and to determine the effect of low-dose warfarin on D-dimer level during vaso-occlusive crisis.. Plasma D-dimer level was measured in 65 blood samples of 37 adult patients with SCD who were hospitalized for vaso-occlusive painful crisis. D-dimer level of patients who were on low-dose warfarin was compared with those patients who were not on any anticoagulation treatment. Analysis of variance (anova) was carried out to determine factors significantly associated with low D-dimer level in patients with SCD. The following factors were included in the anova model; warfarin, homozygous hemoglobin S, history of blood transfusion in past 3 months, hydroxyurea, hemoglobin S%, hemoglobin F%, white blood cell counts, hemoglobin level, platelet count, and plasma fibrinogen level.. Overall median D-dimer level in 65 samples was 2.7 microg fibrinogen equivalent units (FEU)/mL (0.34-4). Patients who were on low-dose warfarin had a median D-dimer level of 0.81 microg FEU/mL (0.34-1.8) compared with 3.1 microg FEU/mL (0.94-4) in those patients who were not on anticoagulation treatment. Using anova to model D-dimer levels, only warfarin was significantly correlated with low D-dimer levels after controlling for other variables.. Patients with SCD during vaso-occulsive painful crisis have an elevated D-dimer level. Low-dose anticoagulation treatment is associated with a significant reduction in the D-dimer levels.

Topics: Adult; Analysis of Variance; Anemia, Sickle Cell; Anticoagulants; Blood Coagulation; Constriction, Pathologic; Female; Fibrin Fibrinogen Degradation Products; Humans; Male; Middle Aged; Vascular Diseases; Warfarin

Patients with sickle cell disease (SCD) experience initial and recurrent venous thromboembolism (VTE) more commonly and at a younger age than the general population, and it confers a higher mortality for patients with SCD. However, limited evidence is available to guide anticoagulant use for VTE treatment in this population. The primary objective of this study is to characterize the effectiveness and safety of direct oral anticoagulants (DOAC) and warfarin for VTE treatment among patients with SCD. This single-center retrospective study includes adult patients with SCD who were diagnosed with VTE. Data was obtained from review of electronic health records for the 6 months after VTE diagnosis. Among the 22 patients treated initially with a DOAC, 6 (27%) developed recurrent VTE, none experienced major bleeding, and 3 (14%) experienced clinically relevant non-major bleeding (CRNMB). Similarly, of 15 patients initially treated with warfarin, 3 (20%) developed a recurrent VTE, 1 (7%) experienced major bleeding, and 2 (13%) experienced CRNMB. Twelve patients received more than one oral anticoagulant during the study period, most commonly due to a recurrent VTE, concern for non-adherence, or subtherapeutic INR. Overall, the incidence of VTE recurrence and bleeding events were similar between groups, but occurred at a higher rate than those found in major clinical trials of anticoagulant agents. Prescribers should continue to individualize therapeutic decision-making regarding oral anticoagulant therapy for VTE treatment for individuals with SCD based on patient-specific factors and anticipated ability to adhere to the drug regimen or required monitoring.

Topics: Adult; Anemia, Sickle Cell; Anticoagulants; Hemorrhage; Humans; Medication Adherence; Precision Medicine; Retrospective Studies; Treatment Outcome; Venous Thromboembolism; Warfarin

Neonatal chondrodysplasia punctata (CDP) is characterized by epiphyseal stippling and midfacial hypoplasia. CDP is usually inherited, but can be acquired because of maternal vitamin K deficiency. We describe an infant with CDP born to a teenager with sickle cell anemia and transfusional iron overload. The mother had severe liver fibrosis, elevated liver iron concentration (34 mg Fe/g), and coagulopathy, but no gestational use of warfarin. Fetal abnormalities were attributed to vitamin K deficiency secondary to liver dysfunction from iron toxicity. Treatment of iron overload among women with sickle cell anemia of childbearing potential is important to avoid possible CDP in newborns.

Topics: Abnormalities, Drug-Induced; Adolescent; Anemia, Sickle Cell; Chondrodysplasia Punctata; Female; Humans; Infant, Newborn; Iron Overload; Liver Diseases; Male; Nasal Bone; Pregnancy; Pregnancy Complications; Transfusion Reaction; Vitamin K Deficiency; Warfarin

Topics: Anemia; Anemia, Sickle Cell; Humans; Warfarin