scholarly journals Haemophilic arthropathy

2014 ◽  
Vol 2 (4) ◽  
pp. 225-227
Author(s):  
Sarath Chandra Mouli V ◽  
Pradeep H
Keyword(s):  
Factor Viii ◽  
Lupus Erythematosus ◽  
Treatment Options ◽  
Left Groin ◽  
Left Hand ◽  
Acquired Haemophilia ◽  
Haemophilic Arthropathy ◽  
Fatal Bleeding ◽  
Coagulant Activity ◽  
History Of

Acquired haemophilia (AH) is extremely rare and potentially fatal bleeding disorder and it is associated with significant morbidity and mortality rate between 7.9% to 22%. Upto 90% of people with AH experience bleeding. It is mainly due to the spontaneous development of auto antibodies that inhibit the coagulant activity of factor VIII (FVIII). Autoimmune disorders (16.6%) like Rheumatoid arthritis, Systemic lupus erythematosus (SLE), Multiple Sclerosis etc., may lead to trigger acquired inhibitors to Factor VIII. In the case report we described a 70-year-old male patient who has history of migratory joint pains and swellings for five months and on physical examination there was an echymotic rash over the left hand elbow and left groin extending into the left thigh and we reemphasize that even though a series of effective treatment options available, a through workup, early diagnosis and early treatment are important.

ACQUIRED HAEMOPHILIA DUE TO FACTOR VIII INHIBITOR WITH SEVERE HAEMORRHAGES IN A 46-YEAR-OLD WOMAN SUCCESSFULLY TREATED WITH CYCLOSPORIN A

1987 ◽  
Author(s):  
Z Boda ◽  
J Hársfalvi ◽  
K Pecze ◽  
K Rak
Keyword(s):  
Factor Viii ◽  
Cyclosporin A ◽  
Factor Viii Inhibitor ◽  
Acquired Haemophilia ◽  
Coagulant Activity ◽  
Recovery Treatment ◽  
History Of ◽  
Viii Inhibitor ◽  
Mucosal Bleeding ◽  
Neurological State

A formerly healthy 46-year-old woman suffering from acquired haemophilia caused by factor VIII antibodies was admitted in an unconscious state following subarachnoid haemorrhage. Treatment with prothrombin complex concentrates (taken as a whole 100 000 U of PCC, home made and 10 OOOUofFEIBA, Immuno) , steroid (Prednison 50 mg/day) and cyclophosphamide (100 mg/day) was only partially successful: neurological state improved but the haemorrhagic tendency remained. Significant haematuria, and skin and mucosal bleeding characterized her clinical picture. In the meantime, signs of non-A non-B hepatitis were observed. After recovery treatment with Cyclosporin A (Sandimmun, Sandoz) was started (250 mg/day per os) together with small dose of Prednison (15 mg/day). No PCC was applied since that time and the partial thromboplastin times (PTT) became gradually shorter. Level of factor VIII inhibitor was 160 Bethesda unit prior and 9 unit after treatment, the duration of that was 60 days till now. Factor VIII coagulant activity (VIIIC) increased from value of less than 1 percent to 13.7 percent.Treatment of acquired haemophilia caused by factor VIII antibodies, particularly in cases with central nervous system bleeding, may be very difficult. History of our patient may indicate that patients resistent to substitution therapy, steroid and cytostatics may response well to Cyclosporin A. Therefore, its use is recommended.

scholarly journals Serial studies in von Willebrand's disease: variability versus "variants"

Blood ◽  
1980 ◽  
Vol 56 (4) ◽  
pp. 712-716 ◽  
Author(s):  
CF Abildgaard ◽  
Z Suzuki ◽  
J Harrison ◽  
K Jefcoat ◽  
TS Zimmerman
Keyword(s):  
Factor Viii ◽  
Repeated Testing ◽  
Laboratory Findings ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Normal Individuals ◽  
Coagulant Activity ◽  
History Of ◽  
Ristocetin Cofactor

The variability of laboratory findings in von Willebrand's disease (vWd) was evaluated by performing serial studies of bleeding time (BT), factor VIII coagulant activity (VIII:C), factor-VIII-related antigen (VIIIR:Ag) and ristocetin cofactor (VIIIR:Rcof) in 50 individuals from 25 families with this disorder. The types of results were characterized from 1 to 16 based on the possible combinations of findings using these four tests. The only patients observed to have consistently abnormal results of all four tests were three individuals with homozygous vWd. Individuals with autosomal dominant vWd were found to have a variety of results and all 16 possible types were observed. Although a consistent pattern was present within some families, others with equivalent history of bleeding demonstrated widely variable types of results. The results within some families, others with equivalent history of bleeding demonstrated widely variable types of results. The results of serial studies of the same tests in 10 normal individuals indicated relative stability, with nearly all values within the usual range of normal, but some independent variation of factor-VIII-related activities was observed. These studies indicate that: (1) the results of BT, VIII:C, VIIIR:Ag, and VIIIR:Rcof vary considerably from time to time in many individuals with vWd, (2) a classification of “variants” of vWd based solely on such studies may be inappropriate, particularly if the tests are not repeated, and (3) repeated testing may be required to establish the diagnosis of vWd in some individuals.

Factor VIII Inhibitors in Mild and Moderate-severity Haemophilia A

1998 ◽  
Vol 79 (04) ◽  
pp. 762-766 ◽  
Author(s):  
C.A. Ludlam ◽  
B.T. Colvin ◽  
F.G.H. Hill ◽  
F.E. Preston ◽  
N. Wasseem ◽  
...  
Keyword(s):  
Factor Viii ◽  
Tolerance Induction ◽  
Haemophilia A ◽  
Missense Mutations ◽  
Bleeding Pattern ◽  
Spontaneous Bleeding ◽  
Familial Predisposition ◽  
Inhibitor Development ◽  
Acquired Haemophilia ◽  
History Of

SummaryTwenty six patients with mild or moderate haemophilia A and inhibitors are described. The inhibitor was detected at a median age of 33 years, after a median of 5.5 bleeding episodes. This usually following intensive replacement therapy. The median presenting inhibitor titre was antihuman 11.6 BU/ml, antiporcine 1.45 BU/ml. Plasma basal factor VIII level declined from a median of 0.08 IU/ml to 0.01 IU/ml following the inhibitor development. This caused spontaneous bleeding in 22 and a bleeding pattern similar to acquired haemophilia in 17. Bleeding was often severe and caused two deaths.The inhibitor disappeared spontaneously, or following immune tolerance induction, in 16 cases after a median of 9 months (range 0.5-46), with a return to the original baseline VIIIC level and bleeding pattern accompanied inhibitor loss. The inhibitor persisted in the remainder of the cases over a median period of 99 months (range 17-433 months) of follow-up. Inhibitors are an uncommon complication of mild haemophilia which frequently persist and may be associated with severe, life-threatening, haemorrhage.Forty-one percent of treated haemophilic family members had a history of factor VIII inhibitors, suggesting a familial predisposition to develop inhibitors in these kindreds. Sixteen patients from 11 families were genotyped. Seven different missense mutations affecting the light chain were detected and two in the A2 domain. Five patients from three families had a mutation causing a substitution of Trp2229 by Cys in the C2 domain which appears to predispose to inhibitor formation since 7 of the 18 affected individuals have a history of inhibitor development.

Doxycycline-induced acquired haemophilia A

2021 ◽  
Vol 14 (10) ◽  
pp. e244748
Author(s):  
Ejaz Shah ◽  
Calvin Abro ◽  
Fawwad Zaidi ◽  
Ruchika Goel
Keyword(s):  
Acute Respiratory Distress Syndrome ◽  
Respiratory Failure ◽  
Factor Viii ◽  
Distress Syndrome ◽  
Therapeutic Agents ◽  
Activated Partial Thromboplastin Time ◽  
Factor Vii ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
History Of

An 80-year-old man with no personal or family history of bleeding, presented to hospital with extensive haematomas and skin bruising after using doxycycline. His basic lab workup was concerning for a coagulopathy with an elevated activated partial thromboplastin time and significant anaemia. Mixing studies and other factor levels were tested that led to the diagnosis of acquired haemophilia A with low factor VIII levels and high factor VIII antibodies. He was started on steroids, but his haemoglobin level continued to drop. Later, during his treatment, he was given multiple therapeutic agents, including cyclophosphamide, rituximab and recombinant factor VII (NovoSeven-R). Gradually factor VIII levels increased and haemoglobin stabilised. The hospital course was complicated by COVID-19 pneumonia leading to acute respiratory distress syndrome; the patient eventually expired due to respiratory failure.

scholarly journals Progressive Intramuscular Haematoma in a 12-Year-Old Boy: A Case of Acquired Haemophilia A

2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Manori Gamage ◽  
Sadeepa Weerasinghe ◽  
Mohamed Nasoor ◽  
A. M. P. W. Karunarathne ◽  
Sashi Praba Abeyrathne
Keyword(s):  
Factor Viii ◽  
Soft Tissues ◽  
Coagulation Factor ◽  
Bleeding Disorder ◽  
Haemophilia A ◽  
Fviii Inhibitor ◽  
Acquired Haemophilia ◽  
High Antibody Titer ◽  
History Of ◽  
The Right

Acquired hemophilia A (AHA) is a rare bleeding disorder due to acquired antibodies against coagulation factor VIII (FVIII). It is rare in children less than 16 years old, and the incidence is 0.45/million/year. An otherwise healthy, 12-year-old boy was admitted to the ward with a history of swelling of the right and left forearms, for 1 day duration. He did not have any history of trauma or bleeding disorder. He had prolonged APPTT level with very high antibody titer against factor VIII. His gene expression for factor VIII was found to be normal. He was managed with FEIBA and recombinant FVII activated complexes and prednisolone 1 m/kg/day regime to control bleeding. AHA is associated with several underlying pathologies such as pregnancy, autoimmune diseases, malignancy, medications and infections; however, up to 50% of reported cases are idiopathic. In contrast to congenital haemophilia A, in which haemarthrosis is the hallmark clinical presentation, patients with AHA mainly bleed in to the skin, muscles, and soft tissues. High mortality rate of more than 20% is either to retroperitoneal or intracranial bleeds. Diagnosis is confirmed on isolated prolongation of activated partial thromboplastin time which does not normalize after addition of normal plasma, reducing the factor VIII levels with evidence of FVIII inhibitor activity. They have normal prothrombin time and platelet functions. Management of AHA involves two aspects, namely, eradication of antibodies and maintaining effective haemostasis during a bleeding episode.

scholarly journals Lung Involvements in Rheumatic Diseases: Update on the Epidemiology, Pathogenesis, Clinical Features, and Treatment

2018 ◽  
Vol 2018 ◽  
pp. 1-19 ◽  
Author(s):  
You-Jung Ha ◽  
Yun Jong Lee ◽  
Eun Ha Kang
Keyword(s):  
Rheumatoid Arthritis ◽  
Risk Factors ◽  
Rheumatic Diseases ◽  
Lupus Erythematosus ◽  
Treatment Options ◽  
Specific Form ◽  
Lung Involvement ◽  
Systemic Lupus ◽  
The Past ◽  
History Of

Lung illness encountered in patients with rheumatic diseases bears clinical significance in terms of increased morbidity and mortality as well as potential challenges placed on patient care. Although our understanding of natural history of this important illness is still limited, epidemiologic knowledge has been accumulated during the past decade to provide useful information on the risk factors and prognosis of lung involvements in rheumatic diseases. Moreover, the pathogenesis particularly in the context of genetics has been greatly updated for both the underlying rheumatic disease and associated lung involvement. This review will focus on the current update on the epidemiologic and genetics features and treatment options of the lung involvements associated with four major rheumatic diseases (rheumatoid arthritis, systemic sclerosis, myositis, and systemic lupus erythematosus), with more attention to a specific form of involvement or interstitial lung disease.

scholarly journals Unusual olecranon mass with ulnar nerve compressive neuropathy caused by a haemophilic pseudotumour

2019 ◽  
Vol 12 (9) ◽  
pp. e231589 ◽  
Author(s):  
Caleb Matthew Yeung ◽  
Philip Blazar
Keyword(s):  
Ulnar Nerve ◽  
Therapeutic Option ◽  
Surgical Excision ◽  
Left Hand ◽  
Left Elbow ◽  
Compressive Neuropathy ◽  
Haemophilic Arthropathy ◽  
Olecranon Bursitis ◽  
History Of

A 55-year-old man with a history of haemophilia A and bilateral haemophilic arthropathy of the elbows presented with an enlarging left elbow mass and worsening paresthesias in the ulnar distribution of the left hand. The mass, originally thought to be olecranon bursitis and treated as such, was found to be due to a haemophilic pseudotumour. The patient underwent successful excision of the haemophilic pseudotumour with concomitant ulnar nerve decompression and had sustained resolution of the pseudotumour and symptoms at 10 years of follow-up. This case demonstrates the need for consideration of haemophilic pseudotumour in the differential diagnosis for olecranon masses and cubital tunnel compressive neuropathy in patients with haemophilia, and highlights the viability of surgical excision as a therapeutic option for the treatment of haemophilic pseudotumours in the elbow.

scholarly journals A case of serious bleeding

2015 ◽  
Vol 5 (2S) ◽  
pp. 15-19
Author(s):  
Irene Ricca ◽  
Marisa Coggiola ◽  
Silvia Destefanis ◽  
Claudio Pascale
Keyword(s):  
Mortality Rate ◽  
Autoimmune Diseases ◽  
Factor Viii ◽  
Coagulation Factor ◽  
The Elderly ◽  
Severe Anaemia ◽  
Severe Bleeding ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
History Of

Acquired haemophilia A (AHA) is a rare disorder with a high mortality rate. It occurs due to autoantibodies against coagulation factor VIII (FVIII) which neutralise its procoagulant function resulting in severe bleeding. This disease may be associated with autoimmune diseases, malignancies, infections or medications and occurs most commonly in the elderly. Diagnosis is based on the isolated prolongation of aPTT which does not normalise after the addition of normal plasma along with reduced FVIII levels. Treatment involves eradication of antibodies and maintaining effective haemostasis during bleeding. We report a case of a 76-year-old patient with a history of haemorrhage with severe anaemia. The article describes difficulties and complexities of clinical and therapeutic management of the patient.

scholarly journals Serial studies in von Willebrand's disease: variability versus "variants"

Blood ◽  
1980 ◽  
Vol 56 (4) ◽  
pp. 712-716 ◽  
Author(s):  
CF Abildgaard ◽  
Z Suzuki ◽  
J Harrison ◽  
K Jefcoat ◽  
TS Zimmerman
Keyword(s):  
Factor Viii ◽  
Repeated Testing ◽  
Laboratory Findings ◽  
Von Willebrand's Disease ◽  
Von Willebrand’S Disease ◽  
Normal Individuals ◽  
Coagulant Activity ◽  
History Of ◽  
Ristocetin Cofactor

Abstract The variability of laboratory findings in von Willebrand's disease (vWd) was evaluated by performing serial studies of bleeding time (BT), factor VIII coagulant activity (VIII:C), factor-VIII-related antigen (VIIIR:Ag) and ristocetin cofactor (VIIIR:Rcof) in 50 individuals from 25 families with this disorder. The types of results were characterized from 1 to 16 based on the possible combinations of findings using these four tests. The only patients observed to have consistently abnormal results of all four tests were three individuals with homozygous vWd. Individuals with autosomal dominant vWd were found to have a variety of results and all 16 possible types were observed. Although a consistent pattern was present within some families, others with equivalent history of bleeding demonstrated widely variable types of results. The results within some families, others with equivalent history of bleeding demonstrated widely variable types of results. The results of serial studies of the same tests in 10 normal individuals indicated relative stability, with nearly all values within the usual range of normal, but some independent variation of factor-VIII-related activities was observed. These studies indicate that: (1) the results of BT, VIII:C, VIIIR:Ag, and VIIIR:Rcof vary considerably from time to time in many individuals with vWd, (2) a classification of “variants” of vWd based solely on such studies may be inappropriate, particularly if the tests are not repeated, and (3) repeated testing may be required to establish the diagnosis of vWd in some individuals.

Sign in / Sign up

Export Citation Format

Share Document