Scurvy presenting as lower limb ecchymoses in the setting of metastatic colorectal cancer.

A 58-year-old woman presented with a 1-week history of lower limb bruising. She had a medical history of recurrent metastatic colon cancer with a sigmoid colectomy and complete pelvic exenteration leading to colostomy and urostomy formation. She had malignant sacral mass encroaching on the spinal cord. This caused a left-sided foot drop for which she used an ankle-foot orthosis. She was on cetuximab and had received radiotherapy to the sacral mass 1 month ago. On examination, there were macular ecchymoses with petechiae on the lower limbs. There was sparing of areas that had been compressed by the ankle-foot orthosis. Bloods showed mild thrombocytopaenia and anaemia with markedly raised inflammatory markers. Coagulation studies consistent with inflammation rather than disseminated intravascular coagulation. She was found to have Klebsiella bacteraemia secondary to urinary source. Skin biopsy showed dermal haemorrhage without vessel inflammation. Vitamin C levels were low confirming the diagnosis of scurvy.

The Application of Thromboelastography in Understanding and Management of Ecchymosis After Total Knee Arthroplasty.

BACKGROUND: Ecchymosis is a common complication after total knee arthroplasty (TKA). However, only a few studies have been conducted to determine the coagulation status of these patients. Using thromboelastography (TEG), a new technique recording the viscoelastic changes of a whole-blood sample, this study was performed to understand the ecchymosis-related coagulation status and its risk factors. METHODS: Seventy-five patients receiving unilateral TKA were recruited in this observational study. Oral rivaroxaban was applied as prophylaxis treatment after surgery. TEG was performed 1 day before surgery and 4 days after surgery. Total blood loss and hidden blood loss were recorded and calculated. Univariate and multivariate analyses were conducted to determine risk factors. RESULTS: Twenty-five patients (33.3%) developed ecchymosis within 2-3 days after TKA. Compared with the baseline before TKA, the α-angle (P = .032) and calculated coagulation index (CI; P = .012) were increased in non-ecchymosis group, whereas in the ecchymosis group, there was no significant difference regarding any variable of postoperative TEG evaluation. The ecchymosis group exhibited more hidden blood loss (P = .007) and total blood loss (P = .011). Value change of CI (OR = 0.666, 95% confidence interval = [0.496, 0.895], P = .007) and hidden blood loss (OR = 1.008, 95% confidence interval = [1.002, 1.013], P = .004) were recognized as the independent risk factors for postoperative ecchymosis. CONCLUSION: In TEG evaluation, the value change of CI and hidden blood loss are independent risk factors of ecchymosis. In view of the relative hypocoagulation status, it is reasonable to stop anticoagulation therapy in patients with excessive ecchymosis. Personalized anticoagulation therapy may be helpful for managing ecchymosis after TKA.

Acquired haemophilia A complicating alemtuzumab therapy for multiple sclerosis.

Alemtuzumab is a highly efficacious therapy used in the treatment of multiple sclerosis (MS), but uncoupling of T and B cell repopulation during immune reconstitution associates with an increasing range of secondary B cell-mediated autoimmune complications. A 34-year-old woman developed Graves' disease 11 months following an initial course of alemtuzumab treatment for MS. Nine months following the second treatment with alemtuzumab, the patient presented with spontaneous intramuscular and subcutaneous haemorrhage due to development of an inhibitory autoantibody to coagulation factor VIII. Acquired haemophilia A (AHA) is an extremely rare complication in patients treated with alemtuzumab. Treatment with rituximab may induce a rapid remission of AHA; however, the patient's high John Cunningham virus (JCV) antibody index and alemtuzumab-induced T cell lymphopenia may lead to an increased risk of progressive multifocal leucoencephalopathy, a potential complication which was unacceptable to the patient.

Acquired hemophilia A in the HIV-infected patient: a case report and literature review.

Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies against factor VIII (FVIII). Spontaneous bleeding in the various sites and severity is the most common clinical presentation. Here, we report a 74-year-old Thai woman with HIV infection who presented with spontaneous ecchymoses. The laboratory revealed isolated activated partial thromboplastin time prolongation with low FVIII activity and a presence of FVIII inhibitor. She was diagnosed with acquired hemophilia A. Corticosteroid monotherapy was the treatment regimen for inhibitor eradication. We demonstrate the clinical course of the rare condition and review the relevant literature.

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 (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.

Unusual bleeds, unusual clots.

Nine unusual bleeding and clotting disorders (or mimickers of such) are described in the format of case presentations, with focus on clinical history, images and diagnostic tests, followed by a discussion of the disease itself and a summarizing clinical teaching point. The disease entities discussed are acquired factor VIII inhibitor, acquired von Willebrand factor inhibitor, haemophilic pseudotumour, Gardner-Diamond syndrome, coumarin-induced skin necrosis, purple toe syndrome, brachiocephalic vein thrombosis with breast enlargement, and leg swelling due to nephrogenic fibrosing dermopathy and lymphoedema. The publication is meant to demonstrate the fascination of clinical coagulation.

[Decreased Quick percentage, acquired factor X deficiency, hemarthrosis and ecchymosis: amyloidosis]. / Verminderter Quickprozentwert, akquirierter Faktor X-Mangel, Gelenksblutung und Hautblutungen: Amyloidose.

A 68-year-old woman suffered from spontaneous hemarthrosis of the right elbow joint in april 1994. Cutaneous ekchymoses had been noted since summer 1993. Prothrombin time was prolonged (Quick percentage 40-50%) and was not corrected by prolonged administration of vitamin K. Coagulation studies showed isolated factor X (FX) deficiency without circulating FX inhibitor. This suggested the diagnosis of systemic amyloidosis which was retrospectively confirmed in small bowel biopsy specimens obtained one year before. Bilateral femoral head necrosis and femoral neck fracture due to amyloidosis, necessitated orthopedic surgery. The patient died four weeks postoperatively after several episodes of bleeding complications. Isolated FX deficiency may be hereditary but should--in the clinical context--also evoke the diagnosis of systemic amyloidosis. The possible bleeding tendency associated with amyloidosis is not attributable to acquired FX deficiency alone, but may also be caused by amyloid deposition in the microvasculature leading to acquired vascular hemorrhagic diathesis.

Acquired factor VIII inhibitor preceding chronic lymphocytic leukemia.

An acquired factor VIII inhibitor was found in an 82-year-old woman who presented with numerous spontaneously appearing ecchymoses. Coagulation studies revealed the presence of a prolonged activated partial thromboplastin time that was not corrected by 1:1 mixture with normal fresh plasma after a 2-h incubation. Factor VIII:C was 4%, and the titer for factor VIII inhibitor was 9 Bethesda units. Three months later, after a retroperitoneal hemorrhage, a lymphocytosis was found in her peripheral blood with morphological and surface immunophenotype characteristics of B-cell chronic lymphocytic leukemia, later confirmed by bone marrow morphological and immunocytochemical examinations. To our knowledge, this is the first report of an autoimmune factor VIII inhibitor associated with chronic lymphocytic leukemia.

Rodenticide-induced coagulopathy in a young child. A case of Munchausen syndrome by proxy.

PURPOSE: To present the diagnosis and management of superwarfarin ingestion, a cause of serious and prolonged coagulopathy. METHODS: Specific identification of the anticoagulant was made by high-pressure liquid chromatography. RESULTS: A 24 month-old child developed bruises and a prolonged prothrombin time (PT) and activated partial thromboplastin time (aPTT) after receiving multiple doses of brodifacoum, a superwarfarin rodenticide. The coagulopathy was treated successfully with large doses of parenteral and oral vitamin K1; fresh frozen plasma was administered as a precautionary measure on two occasions. After the first 10 days of the child's hospitalization, the mother was identified as the source of brodifacoum, exemplifying the behavior described as Munchausen syndrome by proxy. Oral vitamin K1 was initiated and continued in an outpatient setting with tapering doses over nine months, using the PT as a guide for therapy. CONCLUSIONS: This report emphasizes the necessity of recognizing rodenticide poisoning and investigating its source. Frequent monitoring of the PT is essential to prevent hemorrhagic complications due to repeat exposure, inadequate vitamin K1 therapy, or noncompliance.

Coagulation factors in orbital venous vasculitis.

Orbital venous vasculitis has earlier been shown to be associated with spontaneous ecchymoses. With the reservation that platelet function was not investigated and fibrinolysis only partly, this study of the coagulation factors of eight patients with orbital venous vasculitis showed normal findings, suggesting immunologic etiology rather than coagulation deficiency as the cause of the spontaneous ecchymoses.

Fibrinolytic study in a homozygous protein C deficient patient.

The fibrinolytic system was evaluated in a patient with homozygous protein C deficiency as well as in several members of his family with a partial deficiency of this protein. Before anticoagulant therapy the patient showed skin lesions which quickly disappeared after administration of fresh plasma. After anticoagulant treatment, the propositus suffered two clinical episodes of "ecchymotic" lesions, which were controlled with fresh plasma. The patient has remained free of new lesions and other clinical episodes up to the present date. The fibrinolytic activity of both the propositus and his family was normal. The patient's father showed adequate release of tissue plasminogen activator after controlled physical exercise. According to clinical and analytical data from our patient and his family, it is suggested that, in spite of the preservation of the fibrinolytic system in this case, a localized deficiency in fibrinolysis could exist in view of the clinical behaviour of the skin lesions described.

Increased releasable vascular plasminogen activator and a bleeding diathesis.

A 66-year-old man with metastatic prostatic carcinoma treated with high-dose (5 mg) diethylstilbestrol therapy underwent orchiectomy to allow discontinuation of estrogen. An extensive ecchymosis developed postoperatively in an area covered by elastic dressing tape; subsequently, it was learned that the patient had a recent history of frequent ecchymoses with mild trauma that proceeded to vesiculation. His alpha2-antiplasmin level was 132 percent, fibrin split products 4 micrograms/ml, and fibrinogen 293 mg/dl; routine coagulation results were normal. However, the level of releasable vascular plasminogen activator in a specimen drawn prior to surgery was 3.6 CTA units/ml, the highest value ever reported in this laboratory.