Spontaneous Ecchymosis Research Articles

Evaluation of patients admitted to the hematology outpatient clinic for spontaneous ecchymosis: Does it suggest bleeding diathesis?

Background: Spontaneous ecchymosis is a common reason for referral to the hematology outpatient clinic. It is important to determine whether the underlying bleeding is benign or a symptom of diathesis. Methods: The sample of this retrospective study consisted of 119 patients with normal platelet counts who presented to the adult hematology outpatient clinic with spontaneous ecchymosis between September 2021 and August 2022. The site of the ecchymosis, patients’ familial and bleeding histories, and the drugs they have been using were queried. Hemogram and coagulation parameters and peripheral smear findings of the patients were recorded, and their ISTH-SSC (International Society on Thrombosis and Haemostasis Scientific and Standardization Committee) Bleeding Assessment Tool scores were calculated. Results: The median age of the patients, of whom 113 (95%) were female and 6 (5%) were male, was 30 (18-85) years. The ecchymosis was in the extremities and the trunk in 110 (92.4%) and 9 (7.6%) patients, respectively. There was no significant difference between the patients with and without bleeding history in terms of the site of spontaneous ecchymosis, the hemogram parameters and peripheral smear findings. Similarly, the patients with and without factor deficiency have not differed significantly in terms of hemogram parameters and peripheral smear findings. Patients with any factor deficiency had significantly higher ISTH-SSC Bleeding Assessment Tool scores compared to the patients without factor deficiency. Conclusion: The findings of the study revealed that the site of ecchymosis and hemogram and coagulation parameters cannot be used as markers in the general sense, except for the ISTH-SSC Bleeding Assessment Tool scores, which predicted factor deficiency. Another finding of the study worth mentioning is the fact that number of patients with ecchymosis on the trunk among the patients presenting with the complaint of ecchymosis was significantly lower than that of patients with ecchymosis on the extremities.

A Rare Case of an Acquired Isolated Factor VII Deficiency was Discovered in a 23-Year-Old Female Patient

Introduction: Factor VII (FVII) deficiency, a rare bleeding disorder, can manifest as an autosomal recessive congenital or an acquired coagulopathy. Acquired FVII deficiency, although infrequently reported, presents unique challenges in understanding its mechanisms and identifying underlying causes. Case presentation: We present a case of acquired FVII deficiency discovered in a 23-year-old female patient with no apparent underlying disease. The patient exhibited spontaneous ecchymosis and gingival hemorrhage, along with low FVII activity and isolated prolongation of prothrombin time. Extensive laboratory investigations excluded liver dysfunction, familial deficiency, vitamin K deficiency, and inhibitory antibodies. Prompt treatment with Fresh Frozen Plasma (FFP) and bypassing agents resulted in a favorable response and resolution of hematomas. Conclusion: Acquired FVII deficiency was identified with bleeding symptoms in association with prolonged prothrombin time and a low level of FVII activity. In literature, this deficiency has been associated with various conditions such as sepsis, aplastic anemia, stem cell transplantation, and neoplasms, although approximately 14% of cases remain idiopathic. Clinical outcomes remain generally poor, with limited complete remissions reported.

84-Year-Old Female With Easy Bruising

An 84-year-old female presented to the primary care clinic with easy bruising that began approximately 3 months prior. At that time, she noticed a large spontaneous ecchymosis on her left arm. Over the following weeks, she developed atraumatic ecchymoses over the bilateral arms, legs, abdomen, and back. She denied epistaxis, gingival bleeding, or difficulty achieving hemostasis. Review of systems was positive for 15 kg unintentional weight loss over the preceding year, which was corroborated by clinic weights. She denied fevers, night sweats, lymphadenopathy, fatigue, myalgias, chest pain, dyspnea, hematemesis, hemoptysis, hematuria, hematochezia, or melena. CORRECT ANSWERS: 1. e. 2. a. 3. d. 4. e. 5. b.

Un caso insolito di trombocitopenia

A two-and-a-half-year-old child presents with spontaneous ecchymosis. In addition to severe thrombocytopoenia, initial investigations show the presence of neutropoenia and signs of immune-mediated haemolysis (positivity of the direct antiglobulin test). This raises the suspicion of Evans syndrome, since this syndrome is characterized, according to the current definition, by the destruction of at least two medullary cell lines in the absence of other diagnoses. Evans syndrome in many cases can be secondary to various pathological conditions. Investigations are carried out in the infectious, immunological, rheumatological and genetic fields, and lead to the identification of a mutation that is one of the causes of common variable immunodeficiency (CVID). So, on that ground, it can be affirmed that the present case of Evans syndrome is secondary to CVID.

A Case of Acquired Platelet Dysfunction with Eosinophilia in Our Hospital “Omer Nishani”, Gjirokaster

Acquired platelet dysfunction with eosinophilia (APDE) first reported in 1975 is an acquired, transient bleeding disorder of unknown etiology characterized by spontaneous ecchymosis, eosinophilia and platelet dysfunction. This bleeding disorder, otherwise known as “non-thrombocytopenic purpura with eosinophilia”, occurs commonly in indigenous Southeast Asian children. Cases of APDE occur rarely outside this endemic area. This syndrome runs a benign course and patients can recuperate spontaneously from the bleeding episodes within 6 months to a year. The aim of our case report is to highlight the importance of recognizing this little known bleeding disorder in our daily clinical practice. We reported the first documented case of a 7 year old girl managed in our hospital “Omer Nishani”, with clinical features characteristic of APDE. The patient was in good health, presented with spontaneous bruising on the extremities with no organomegaly. Marked eosinophilia and prolonged bleeding time with normal platelet count were noted on laboratory work-up. The patient was started on antiparasitic therapy despite negative stool results, and subsequent follow-up after 3 weeks showed resolution of ecchymosis and eosinophilia. Awareness of this benign bleeding disorder, which clinically mimics Idiopathic Thrombocytopenic Purpura (ITP) would help to diagnose and treat early, and lessen parental anxiety.

Immune Thrombocytopenic Purpura presenting as paraneoplastic syndrome in a Dedifferentiated Liposarcoma: A case report

Introduction: Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder characterized by isolated low platelet counts, that occurs because of increased platelet destruction by the production of autoantibodies against platelet antigens. ITP secondary to solid neoplasia is rare. The treatment consists of pharmacological treatment to improve thrombocytopenia and the treatment of the underlying malignancy. Case Report: A 72-year-old woman was admitted in the emergency department with petechial lesions and spontaneous ecchymosis with about three weeks of evolution. Analytically confirmed severe thrombocytopenia, without anemia and normal leukogram, and positive direct antigliadin test, admitting thrombocytopenic purpura. In the subsequent etiological study, a nodular formation was identified in the right para renal area, later biopsied confirming the diagnosis of dedifferentiated retroperitoneal liposarcoma. Despite an initial favorable response to corticosteroid therapy, there was persistence of low platelet count despite first-line therapy (corticosteroids and immunoglobulin), with the need to associate second-line drugs (rituximab and Eltrombopag). There was remission of ITP after complete tumor excision. Conclusion: The association of immune thrombocytopenia with dedifferentiated liposarcoma is very rare, with only one previous case described in the medical literature. The treatment of an underlying malignancy may lead to resolution of thrombocytopenia, which is why, although the association of ITP with solid tumors is very uncommon, it is important to screen for solid tumor in the etiological study of ITP. KEYWORDS Immune thrombocytopenia, dedifferentiated retroperitoneal liposarcoma, paraneoplastic syndrome.

A familial case of MYH9 gene mutation associated with multiple functional and structural platelet abnormalities

Mutations in the MYH9 gene result in macrothrombocytopenia often associated with hemorrhages. Here, we studied the function and structure of platelets in three family members with a heterozygous mutation R1933X in the MYH9 gene, characteristic of closely related disorders known as the May-Hegglin anomaly and Sebastian syndrome. The examination included complete blood count, blood smear microscopy, platelet flow cytometry (expression of P-selectin and active integrin αIIbβ3 before and after activation), the kinetics of platelet-driven contraction (retraction) of blood clots, as well as scanning/transmission electron microscopy of platelets. Despite severe thrombocytopenia ranging (36–86) × 109/l, none of the patients had hemorrhages at the time of examination, although they had a history of heavy menstruation, spontaneous ecchymosis, and postpartum hemorrhage. Flow cytometry showed background platelet activation, revealed by overexpression of P-selectin and active αIIbβ3 integrin above normal levels. After TRAP-induced stimulation, the fractions of platelets expressing P-selectin in the proband and her sister were below normal response, indicating partial platelet refractoriness. The initiation of clot contraction was delayed. Electron microscopy revealed giant platelets with multiple filopodia and fusion of α-granules with dilated open canalicular system, containing filamentous and vesicular inclusions. The novel concept implies that the R1933X mutation in the MYH9 gene is associated not only with thrombocytopenia, but also with qualitative structural and functional defects in platelets. Platelet dysfunction includes impaired contractility, which can disrupt the compaction of hemostatic clots, making the clots weak and permeable, therefore predisposing patients with MYH9 gene mutations to the hemorrhagic phenotype.

Utility of Von Willebrand Testing during Acute Bleeding Episodes

Background: The diagnosis of mild bleeding disorders such as von Willebrand disease (VWD) poses many diagnostic challenges. Several patient-specific factors including physiologic, inflammation, anemia and medications can affect VWD testing and lead to falsely elevated values. Given that the physiologic stress may cause falsely normal levels of VW factor and associated tests, determining the appropriate timing for testing remains an area of debate. Testing is often performed during inpatient or emergency department (ED) visits when patients present for bleeding complaints, although these results may be skewed by stress or illness. Initial normal results may falsely reassure a patient and their health care provider, preventing them obtaining repeat testing or pursuing outpatient hematological evaluation. Further study is needed to determine the reliability of VWD evaluation when obtained during episodes of acute bleeding. Methods: This single-center retrospective cohort study included subjects who were evaluated for acute bleeding complaints in the UPMC Children's Hospital of Pittsburgh ED or inpatient units who had VWD testing sent during that visit over a five-year period. Bleeding complaints included were epistaxis, heavy menstrual bleeding, spontaneous ecchymosis, post-procedural hemorrhage or intracranial hemorrhage. VWD test results including von Willebrand antigen (VWF:Ag), factor VIII (FVIII) and ristocetin co-factor (VWF:RCo) levels obtained at the time of ED or inpatient care were compared with those obtained in outpatient follow-up visits. T-tests and Wilcoxon rank sum tests were used to compare VWD test levels across the two time points. Results: Over a 5-year period, 144 patients had VWD testing completed during ED or inpatient evaluations for acute bleeding complaints. The population was primarily Caucasian (71%) with a median age of 9 years old. Fifty-two percent of the population was male. Epistaxis and post-tonsillectomy/adenoidectomy bleeding were the most common presenting complaints, making up 47 and 22% of the population, respectively. Other presenting bleeding complaints included spontaneous bruising (18%), menorrhagia (6%), post-procedural hemorrhage of a digestive organ (4%), and nontraumatic intracranial hemorrhage (3%). Six subjects (4%) met laboratory criteria for a diagnosis of VWD during an acute bleeding episode. Twenty-four percent of the total population was evaluated subsequently by outpatient hematology, and 23% of patients had repeat VWD testing performed. Median levels of VWD tests obtained at initial presentation were 116% for VWF:Ag, 112% for FVIII, and 85% for VWF:RCo, and were not statistically different from those obtained on repeat testing in outpatient follow up. An additional two patients that were not initially diagnosed with VWD met laboratory criteria for VWD on repeat outpatient testing. Conclusions: Among children presenting for emergency or inpatient care for acute bleeding episodes, VWD testing was unlikely to be diagnostic. Interestingly, our study did not find that median VWF:Ag and FVIII levels were higher during acute bleeding as has been suggested in other studies. A minority of patients who were evaluated in the hospital for acute bleeding were subsequently evaluated by hematology, which may have limited these findings. It is possible that obtaining VWD testing at the time of an acute bleeding episode may provide false reassurance to a patient, thus decreasing hematology referrals and follow up. Based on these results, we recommend that for patients evaluated in the ED with mild mucocutaneous bleeding who warrant a bleeding disorder evaluation, VWD testing be postponed until the patient is seen in steady-state by hematology.

Report of two cases with autoerythrocyte sensitization syndrome

Auto-erythrocyte Sensitization Syndrome, also known as Gardner Diamond syndrome, is characterized by spontaneous painful and repetitive purpura. Here, we report two women aged 14 and 18 years who were diagnosed with auto-erythrocyte sensitization syndrome by typical laboratory, clinical, histopathological features and intradermal skin test. Burning and itching were the major complaints associated with spontaneous ecchymosis. The frequency of attacks in the patient using a specific serotonin re-uptake inhibitor due to migraine was significantly higher than the patient who did not receive psychiatric treatment. It was concluded that careful psychiatric evaluation is important for effective treatment of the disease.

Reversible Right Heart Failure in Scurvy Rediscovery of an Old Observation.

A previously healthy 50-year-old woman presented with 3 months of progressively worsening shortness of breath associated with extreme fatigue, anorexia, a 5 to 10 pound weight loss, yellowing of her eyes, heavy menstrual bleeding, and a lower extremity rash. She denied recent trauma. She had never been admitted to the hospital before and had no history of surgical procedures. She took no medications. There was no significant family history. She was a housewife who had worked in the past as a secretary and had never smoked, drank alcohol, or used recreational drugs. Physical examination revealed blood pressure 91/56 mm Hg, heart rate 92/min, respiratory rate 20/min, and temperature 37°C. Oxygen saturation was 85% on room air. Her skin and conjunctiva were pale, and scleral icterus was present. Her neck veins were distended to her earlobes when sitting, and there was massive hepatomegaly. A nonconfluent, nonblanching petechial rash was noted on her anterior legs (Figure 1A and 1B), and there was a large ecchymosis on her thighs and buttocks (Figure 2). Figure 1. A and B , The petechial rash spreading from the dorsal aspects of her feet up to her knees. Figure 2. Spontaneous ecchymosis on her thighs and buttocks. Laboratory data showed severe normocytic anemia with hemoglobin 3.4 g/dL, hematocrit 12%, mean corpuscular volume 90 fL, reticulocyte count 9.7%, serum iron 9 µg/dL, total iron binding capacity 271 …

Medication Error When Switching from Warfarin to Rivaroxaban Leading to Spontaneous Large Ecchymosis of the Abdominal and Chest Wall

Non-vitamin K oral anticoagulant (NOAC) therapy may be inappropriate if prescription was incorrect, the patient’s physiological parameters change, or interacting concomitant medications are erroneously added. The aim of this report was to illustrate inappropriate NOAC prescription in a 78-year-old woman with non-valvular atrial fibrillation and borderline renal dysfunction who was switched from warfarin to rivaroxaban and subsequently developed bruising with hemorrhagic shock and acute on chronic renal failure. Administration of 4-factor prothrombin complex concentrate effectively reversed coagulopathy and stopped bleeding. Retrospective determination of circulating plasma levels of rivaroxaban and warfarin confirmed that excessive anticoagulation was likely due to warfarin that the patient probably continued to take although rivaroxaban was initiated. Pharmacodynamic interaction between rivaroxaban and warfarin may not only be additive but synergistic. In patients at high risk of complications, judicious prescribing and dosing of NOACs, and regular monitoring of concomitant medications and renal function are highly recommended.

Multiple myeloma-associated skin light chain amyloidosis: A case of misdiagnosis.

The present study reports the case of a 42-year-old male with multiple myeloma (MM)-associated skin light chain amyloidosis who presented with skin purpura as the initial symptom, which was misdiagnosis as Henoch-Schönlein purpura nephritis prior to admission to the Second Xiangya Hospital (Changsha, Hunan, China). The patient presented with purpura, papules petechiae and spontaneous ecchymosis, which was located scattered around the neck, chest and limbs, accompanied by a small amount of bleeding in the conjunctival and oral mucosa, and a swollen tongue. Upon laboratory examination, the serum immunological change showed increased serum immunoglobulin G and λ light chain levels, and a urine Bence Jones protein level of >1 g/24 h. This was accompanied with an abnormal result for immunofixation electrophoresis, and positive staining with Congo red showing apple-green birefringence in skin biopsy specimens. Thus, the patient was diagnosed with MM-associated skin amyloidosis with the initial symptom of skin purpura. Following treatment with chemotherapy consisting of prednisone and bortezomib, the skin lesions markedly improved. The present study indicates that the presentation of skin purpura in systemic amyloidosis associated with MM may be an important aid in the diagnosis and direct treatment of this disease in the clinic.

Massive Bleeding as the First Clinical Manifestation of Metastatic Prostate Cancer due to Disseminated Intravascular Coagulation with Enhanced Fibrinolysis.

Disseminated intravascular coagulation (DIC) is the most frequent coagulation disorder associated with metastatic prostate adenocarcinoma. However, DIC with enhanced fibrinolysis as an initial presentation of prostate cancer is extremely rare. The appropriate treatment to control bleeding in these situations is challenging, controversial, and based on isolated case reports in the literature. A 66-year-old male presented at the emergency department with acute severe spontaneous ecchymoses localized to the limbs, laterocervical hematoma, and hemothorax. Prostate specific antigen level was 385 μg/L, bone scintigraphy revealed multiple bone metastases, and prostate biopsy confirmed adenocarcinoma (Gleason 9; 4 + 5). Laboratory investigation showed a pattern of enhanced fibrinolysis rather than the more common intravascular coagulation mechanism. Epsilon aminocaproic acid in monotherapy was initiated with a clear and rapid control of bleeding manifestations. This rare case of massive bleeding due to DIC with enhanced fibrinolysis as the first manifestation of prostate cancer suggests that in selected cases where the acute bleeding dyscrasia is clearly associated with a dominant fibrinolysis mechanism it is possible to use an approach of monotherapy with antifibrinolytics.

Diagnosis of Cardiac Amyloidosis by Skin Lesions

In June 2011, a 74-year-old man was admitted for recurrent heart failure. He had hypertension, diabetes mellitus, renal failure due to nephrosclerosis and diabetic nephropathy, prostate cancer, tuberculosis, and gouty arthritis, and had received a parathyroidectomy. In 2009, he had received a mechanical prosthesis to treat aortic stenosis. Cardiac auscultation was rhythmic, with no murmurs, and with normal prosthetic sounds. The patient presented petechiae, purpura, papules, and spontaneous ecchymosis at the eyelids, groin, neck, and axillas (Figure 1), with onset after initiation of acenocoumarol in 2009. The electrocardiogram demonstrated sinus rhythm with a long P-R interval, right bundle branch block with left anterior hemiblock, and low voltage, consistent with infiltrative cardiomyopathy (Figure 2). The echocardiogram demonstrated left ventricular hypertrophy with increased echogenicity (Figures 3A and B), ejection fraction of 45%, and a restrictive pattern on pulsed Doppler (Figure 3C: increased E/A ratio) and tissue Doppler (Figure 3D: decreased E’ and A’ peaks). Examination of skin biopsy specimens with Congo red stain showed amyloidosis secondary to monoclonal gammopathy, with positive immunohistochemistry for lambda light chains. He was treated with melphalan and dexamethasone, which were discontinued because of a favorable hematological response, with normalization of serum free light chain concentrations. Thereafter, he was hospitalized several times for heart failure and ultimately died of this cause in 2013. The purpura, petechiae, and ecchymosis were due to intracutaneous bleeding, resulting from a fragile vascular status secondary to amyloid infiltration. This is sometimes the first and only sign of systemic amyloidosis. Although these features are not specific to this disease, they may be an aid to its diagnosis and prompt treatment in the appropriate clinical context. Figure 3. Figure 1. I

Spontaneous Periocular Ecchymosis in Children: Differential Diagnosis and Current Trends in Evaluation and Management

While periocular ecchymosis commonly develops following surgery or traumatic injury to the orbit, the spontaneous appearance of periocular ecchymosis in children can indicate the presence of life-threatening conditions including pediatric malignancies (neuroblastoma, rhabdomyosarcoma, leukemia) and hematologic disorders (aplastic anemia, thrombocytopenia). Vascular malformations (capillary hemangioma, lymphangioma, orbital varix), inflammatory conditions (orbital myositis, amyloidosis), pertussis, and Blue rubber bleb nevus syndrome are benign differential considerations with visual complications in certain instances. Since spontaneous periocular ecchymosis (SPE) can be encountered by pediatric subspecialists both within and outside ophthalmology, the authors present a review of the current literature integrating the clinical features, latest diagnostic investigations, and updates in management for the entities that cause spontaneous periocular ecchymosis in children. A comprehensive and current understanding of the differential diagnosis elicited by this unique ocular finding will aid the clinician in managing long-term visual consequences and coordinating with appropriate pediatric subspecialists.

Ibrutinib (PCI 32765) Rapidly Improves Platelet Counts in Chronic Lymphocytic Leukemia / Small Lymphocytic Lymphoma (CLL/SLL) Patients and Has Minimal Effects On Platelet Aggregation

AbstractAbstract 1789 INTRODUCTION:Ibrutinib (PCI 32765) is an orally administered covalent inhibitor of Bruton's Tyrosine Kinase (BTK). Ibrutinib has significant activity in chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and is typically well tolerated (Byrd ASCO 2011, O'Brien ASH 2011). Rarely serious bleeding in patients concurrently on oral anticoagulation has been reported but was not related to thrombocytopenia (O'Brien ASH 2011). However, grade 1 or 2 ecchymosis/contusion is a frequent adverse event in patients on ibrutinib. In addition to being essential for B cell receptor signaling BTK is also involved in the signaling of the glycoprotein (GP)VI and GPIV von Willebrand (vW) receptors (Liu, Blood 2006). Thus, it is possible that ibrutinib could increase the bleeding risk by interfering with thrombus formation. In addition, lymphoproliferative disorders and some drugs have been associated with acquired vW-disease (AvWD). METHODS AND PATIENTS:In an ongoing single center, open label phase II trial we treat CLL/SLL patients with ibrutinib 420 mg daily on 28 day cycles (NCT01500733). We measured platelet (PLT) function on the PFA-100 instrument, vW-factor (vWF) antigen levels and activity (vWF-Ag/vWF-Act), and factor VIII (FVIII) on baseline, days 2 and 28. Here we report on effects of ibrutinib on platelet counts and function in 25 patients who completed >2 cycles. RESULTS:PLT counts prior to treatment ranged from 36 k/μl to 256 k/μl with a median of 102 k/μl. Twelve (48%) patients had a pre-treatment PLT count <100 k/μl. Median PLT counts for days 14, 28, and 56 increased to 140, 137, and 135 k/μl, respectively (P<.01). 76% of patients showed an increase after only 2 weeks on drug (median increase 25 k/μl (range 4–183 k/μl) that was sustained at subsequent timepoints. On day 14, 6 patients (24%) had a decrease in PLT count by a median of 13 k/μl from baseline; of these, 3 had a pre-treatment PLT count of <100 k/ul and 1 developed grade III thrombocytopenia (42 k/μl) that resolved to >100 k/μl by day 56. 20% (5 of 25) of patients reported grade 1 spontaneous ecchymosis with no correlation to platelet count, PFA testing, or vWF measurements. Of note we performed lymph node core biopsies in 35 patients taking ibrutinib with minimal bruising. Only 2 patients had more extensive local bruising/ecchymosis at the biopsy site.Test (normal range)baselineday 2day 28PLT count (161-347 k/μl)102140137PFA-100 EPI (86-154 sec)109110110PFA-100 ADP (73-129 sec)747979vWF-ACT (52-156 IU/dL)182nd99vWF-AG (50-197 IU/dL)158nd101FVIII (41-184 IU/dL)181nd157All values are median, nd = not doneIn 19 patients PFA-100 measurements of epinephrine (EPI) and adenosine diphosphate (ADP) stimulated platelet aggregation times were available (test requires PLT count >100 k/μl). Median changes in closure times with EPI and ADP on treatment were not significantly different from baseline (See table). Four (21%) patients started with abnormally prolonged EPI closure times (one on aspirin, one on ibuprofen; discontinued with the start of ibrutinib) which resolved by day 28 in 3 and decreased in 1. Three (16%) patients had a prolongation of EPI closure times on day 2 that resolved by day 28 in 2 and decreased in 1. All closure times on ADP were low or normal. No patients with abnormal PFA testing demonstrated spontaneous ecchymosis. From baseline to day 28 vWF-Act, vWF-Ag and FVIII decreased (P<0.05; n=24). All 3 values were high normal to elevated prior to treatment and decreased to normal on treatment. CONCLUSION:This preliminary report does not identify any significant ibrutinib effect on platelet function. PLT counts improved rapidly in the majority of patients and when seen transient decreases have been minimal. Three patients (16%) developed an abnormal reading in PLT function tests on treatment but none developed spontaneous echymosis or bleeding. The observed normalization of mildly elevated baseline levels of vWF and FVIII seems most consistent with a reduction in acute phase reactants and there was no evidence for AvWD on ibrutinib. The apparent functional tolerance of BTK inhibition in platelets is likely attributable to redundancy in the affected signaling pathways.This work was supported by the Intramural Research Program of NHLBI, NIH. We thank our patients for participating in these research studies. Disclosures:No relevant conflicts of interest to declare.

Hémophilie A acquise découverte au cours de la grossesse : à propos d’un cas et revue de la littérature

IntroductionAcquired hemophilia A (AH) is a rare hemorrhagic disorder, secondary to the occurrence of factor VIII inhibitor. In young patients, this disorder is commonly observed during the post-partum period, and has been rarely documented in the prepartum. We report a new case of a prepartum AH and review literature data. Case reportAn isolated prolongation of the activated partial thromboplastin time (APTT) was fortuitously discovered in a 31-year-old pregnant women, with spontaneous ecchymosis of her lower limbs few days prior to delivery. Coagulation tests revealed decreased factor VIII activity (18%) and the presence of factor VIII inhibitor (1,4Bethesda unit). In order to eradicate the autoantibody, the patient was first treated with prednisone and then with rituximab. ConclusionPrepartum factor VIII inhibitors need to be precociously recognized to allow prophylactic management of the delivery bleeding.

Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus; review of literature

Acquired haemophilia A, secondary to systemic lupus erythematosus (SLE) is a rare bleeding diathesis. Here we report a 37-year-old woman with autoimmune hepatitis who developed SLE and acquired haemophilia caused by factor VIII inhibitors. She presented with spontaneous ecchymosis and haematuria. There were a prolongation of the activated partial thromboplastin time, reduced factor VIII activity and a high titer of FVIII inhibitors. Therapeutic regimen was started with intravenous methylprednisolone pulse, continued with prednisolone, intravenous pulse cyclophosphamide and fresh frozen plasma. After 8 weeks, factor VIII inhibitor assay was negative.

Severe Scurvy After Gastric Bypass Surgery and a Poor Postoperative Diet

After bariatric gastric bypass surgery patients are at risk of developing micronutrient deficiencies. If gastric bypass surgery is followed by a vitamin deficient diet the patients have a risk of developing vitamin-C deficiency. When spontaneous ecchymosis is observed in the skin, in at-risk patients, scurvy must be considered. When treated with large doses of vitamin-C the symptoms of scurvy rapidly improve even if the patient has developed multiple organ dysfunction syndrome.KeywordsScurvy; Gastric bypass surgery; Multiorgan dysfunction

Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus

Acquired haemophilia A, secondary to systemic lupus erythematosus (SLE), is a rare bleeding diathesis. Here we report a 37-year-old woman with autoimmune hepatitis who developed SLE and acquired haemophilia caused by factor VIII (fVIII) inhibitors. She presented with spontaneous ecchymosis and haematuria. There were a prolongation of the activated partial thromboplastin time, reduced fVIII activity and a high titre of fVIII inhibitors. Therapeutic regimen was started with intravenous methylprednisolone pulse, continued with prednisolone, intravenous pulse cyclophosphamide and fresh frozen plasma. After 8 weeks, fVIII inhibitor assay was negative.