Malignancy in patients with haemophilia: a review of the literature

Dunn, Amy L.

doi:10.1111/j.1365-2516.2009.02128.x

Cited by 34 publications

(35 citation statements)

References 60 publications

(74 reference statements)

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“…Many types of malignancy have been reported in persons with haemophilia, irrespective of infection with HIV and hepatitis C; yet prevalence and incidence rates compared with the general population remain unknown. In a recent retrospective literature review of cancer in patients with haemophilia, conducted through Medline articles published between January 1966 and July 2009, 32 cases of leucaemia were identified as well as 159 malignant solid tumours [1]. Specific incidence and prevalence rates could not be calculated due to the limited nature of the information available in the reports.…”

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confidence: 99%

“…A multidisciplinary approach to supportive care, including a specialist for treatment of haemophilia, appears warranted. Haemophilia is a lifelong and most commonly encountered inherited bleeding disorder that has significant implications both financially and socially, particularly in under-resourced settings [1]. Although, data on the incidence of haemophilia in developing world are not available, it is estimated that approximately 80% of the haemophiliacs live in developing countries, mainly Asia, Africa and South America [2].…”

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confidence: 99%

“…About two-thirds of haemophiliacs suffer from severe disease [1]. For a low income country like Pakistan, haemophilia is a high cost disease as patient care differs substantially compared with that given in the developed world.…”

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Cord blood hematopoietic stem cell transplantation in an adolescent with haemophilia

et al. 2012

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Haemophilia A results from a congenital deficiency of clotting factor VIII and has an incidence of approximately 1 in 5000 live male births [1]. Patients with access to modern haemophilia care now have improved life expectancy, and will probably experience age-related diseases such as cancer. Many types of malignancy have been reported in persons with haemophilia, irrespective of infection with HIV and hepatitis C; yet prevalence and incidence rates compared with the general population remain unknown. In a recent retrospective literature review of cancer in patients with haemophilia, conducted through Medline articles published between January 1966 and July 2009, 32 cases of leucaemia were identified as well as 159 malignant solid tumours [1]. Specific incidence and prevalence rates could not be calculated due to the limited nature of the information available in the reports.Hematopoietic stem cell transplantation (HSCT) is today an established treatment strategy not only for cancer but also for cure of inborn errors of metabolism or immune system [2]. Although HSCT is not considered a therapeutic option for patients with haemophilia, we describe the clinical course and the outcome of an adolescent with haemophilia in whom HSTC was applied for treatment of relapsed anaplastic large cell lymphoma (ALCL).A. K. was diagnosed with haemophilia A at 18 months in his country (Albania), and treated with blood transfusion and cryoprecipitate. At the age of 6 years, following immigration in Italy, he was put on FVIII therapy. At the age of 12 years he developed a painful scapular mass. Following MR and CT imaging study, he underwent complete resection of the tumour; histological diagnosis of extraosseous EwingÕs sarcoma was made. He was treated, according to the ISG-SSG IV protocol, chemotherapy followed by consolidation with autologous HSCT [3]. Eighteen months after completion of chemotherapy, he developed systemic lymphadenomegaly; histological diagnosis of anaplastic large cell lymphoma (ALCL) was made, and treatment according with the EICNHL protocol for relapsed ALCL was started [4]. After three chemotherapy blocks, he was given highdose chemotherapy with allogeneic HSCT for consolidation of second remission. The conditioning regimen included: Thiotepa 10 mg/kg, Etoposide 40 mg/kg and TBI 12 Gy. The source of HSC was an unrelated cord blood unit; HLA match was 4/6; a total of 2 · 10 5 /kg CD34 cells were infused. Due to his co-morbidity, during the entire transplant phase platelets were infused every other day, aiming at count >30 000/mm 3 , as well as rhFVIII, 1000 IU every 12 h. On day +12, he developed Pseudomonas aeruginosa and Klebsiella pneumonia septicemia. Stable engraftment was documented for neutrophils (>500/ mm 3 ) since day +30, and for platelets (>50 000/mm 3 ) since day +42. On day +44, he was readmitted for hemorrhagic cystitis treated with hyperhydration. On day +65, VNTR analysis documented complete donor chimerism. His clotting tests, including FVIII plasma levels, were similar to his pretransplant...

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Cord blood hematopoietic stem cell transplantation in an adolescent with haemophilia

et al. 2012

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“…Malignancies reported in hemophilic patients are usually associated with HIV, and hepatitis B and C infection. The incidence of non-infection-related malignancy is not known due to the limited quantity of data in the literature [1]. Herein we report a boy with hemophilia A that was followed-up with the initial diagnosis of a neck hematoma; examination of a subsequent excisional biopsy specimen resulted in the diagnosis of nonHodgkin's lymphoma (NHL).…”

Section: To the Editormentioning

confidence: 99%

Non-Hodgkin’s lymphoma in a hemophilic patient with a traumatic hematoma

Özdemir

Yurt²,

Turkan³

et al. 2011

Turk J Hematol

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To the editor,Hemophilia A, the most common severe inherited bleeding disorder, is characterized by FVIII deficiency. The use of recombinant FVIII products and improved purity of plasma-derived concentrates have decreased the risk of HIV, and hepatitis B and C virus transmission in patients with hemophilia. Malignancies reported in hemophilic patients are usually associated with HIV, and hepatitis B and C infection. The incidence of non-infection-related malignancy is not known due to the limited quantity of data in the literature [1]. Herein we report a boy with hemophilia A that was followed-up with the initial diagnosis of a neck hematoma; examination of a subsequent excisional biopsy specimen resulted in the diagnosis of nonHodgkin's lymphoma (NHL).A 12-year-old boy with mild hemophilia A (FVIII coagulant activity 7%; vWF Ag: 78%; Ricof: 72%) had been followed-up at another center since he was 5 years old. Six months before presentation at our institution the patient had a tooth extracted and developed a mass in the neck. Initially, a muscle hematoma was suspected. As the mass enlarged, needle aspiration was performed, and the result showed reactive hyperplasia. The patient was referred to our clinic for further investigation.The patient did not have symptoms suggesting lymphoma or any other malignancy. Physical examination was normal, except for a 2×1.5-cm soft mass in the neck. Full blood count, peripheral blood smear, sedimentation rate (7 mm/h), and lactate dehydrogenase (LDH: 184 IU/L) were normal. Cervical magnetic resonance imaging (MRI) showed a heterogenous solid mass between the submandibular gland and sternocleidomastoid muscle. Thoracic and abdominal computed tomography (CT) results were normal. Positron emission tomography (PET) showed hypermetabolic lymphadenopathies in the left inferior cervical and supraclavicular fossa. Excisional biopsy under factor replacement showed T cell-rich diffuse B cell NHL. There was no bone marrow or cerebrospinal fluid involvement, and he was classified as stage 2 NHL. Viral serology, including HIV, and hepatitis B and C viruses was negative. The patient was treated according to the BFM-NHL 95 protocol and did not have any severe bleeding episodes during chemotherapy. Moreover, no complications occurred during cerebrospinal fluid tap or bone marrow aspiration/biopsy.

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“…A person with coagulation defect may experience a protective effect against the spread of cancer. On the other hand, infections of HCV and HIV can increase the risk of cancer development, but data on this important issue is scarce .…”

Section: Introductionmentioning

confidence: 99%

Increased cancer risk in patients with haemophilia A: a nationwide population‐based 14‐year study in Taiwan

Liu

Teng

et al. 2014

Haemophilia

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Haemostasis is associated with the development and dissemination of cancer. Whether cancer incidence is increased in haemophiliacs remains uncertain; thus, we aimed to further examine this issue. By using data from the National Health Insurance Research Database in Taiwan, we obtained a cohort of 683 patients with haemophilia A, and compared the incidence rate ratio (IRR) of cancer in this cohort with an age- and sex-matched control of 6830 patients. The log-rank test was used to compare Kaplan-Meier curve of the cumulative cancer incidence between two cohorts. Cox regressions were used to identify independent risk factors of cancer in the study patients. The cancer incidence of patients with haemophilia A was significantly higher compared to the control group (IRR 1.95, 95% CI 1.18-3.09, P = 0.008) during the 14-year follow-up period. The non-lymphoma and non-liver cancer incidence in the haemophilia A cohort remained higher than that of the matched control (P = 0.050 by the log-rank test). The multivariate Cox proportional hazards analysis indicated that age (per year, HR 1.09, 95% CI 1.06-1.12, P < 0.001) was the only significant risk factor for cancer development in haemophilia patients. Patients with haemophilia A had higher cancer incidence than the age- and sex-matched patients, especially for the elderly. With increasing life expectancy for haemophiliacs, physicians should be aware of their cancer development.

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Malignancy in patients with haemophilia: a review of the literature

Cited by 34 publications

References 60 publications

Cord blood hematopoietic stem cell transplantation in an adolescent with haemophilia

Cord blood hematopoietic stem cell transplantation in an adolescent with haemophilia

Non-Hodgkin’s lymphoma in a hemophilic patient with a traumatic hematoma

Increased cancer risk in patients with haemophilia A: a nationwide population‐based 14‐year study in Taiwan

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