Two cases of acquired haemophilia A associated with chronic myelomonocytic leukaemia

Uaprasert, Noppacharn; Wongrakpanich, Supakanya; Rojnuckarin, Ponlapat

doi:10.1097/mbc.0b013e328360d038

Cited by 6 publications

(7 citation statements)

References 9 publications

(7 reference statements)

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“…We considered that the intracranial haemorrhage and serious bleeding tendency was mainly due to CML, which caused infiltration of leukaemia cells into the bone marrow, leading to thrombocytopenia and platelet function inhibition, because the FVIII level was not seriously decreased during the emergency presentation and FVIII antibody was negative. This is in contrast to acquired haemophilia A, associated with the presence of antibody [3]. Therefore, transfusion of platelets may be more effective than FVIII and should be more aggressive in patients such as the one reported herein.…”

Section: Discussionmentioning

confidence: 64%

“…The majority of the literature describing co-existing haemophilia and leukaemia describes patients with haemophilia who developed HIV-associated malignancies (e.g. T-cell leukaemia associated with human T-cell leukaemia virus) [2] or patients with CML developing an acquired haemophilia due to the development of an FVIII inhibitor [3]. The occurrence of CML with established haemophilia A has never been reported, and presents a unique management challenge for which there is no consensus in the literature, especially when the CML is exacerbated.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

The first case report of a patient with simultaneous haemophilia A and accelerated phase chronic myelogenous leukaemia

Zheng

Shi

et al. 2015

Haemophilia

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Section: Discussionmentioning

confidence: 64%

Section: Discussionmentioning

confidence: 99%

The first case report of a patient with simultaneous haemophilia A and accelerated phase chronic myelogenous leukaemia

Zheng

Shi

et al. 2015

Haemophilia

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“…In this report, we present one case of AHA caused by CMML. Only three similar cases of AHA associated with CMML have been reported before in the literature [6,7]. The data of the patient are shown in Table 1.…”

Section: Discussionmentioning

confidence: 79%

Chronic myelomonocytic leukaemia associated with acquired haemophilia A: case report and literature review

Liang

Wang

et al. 2015

Haemophilia

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Chronic myelomonocytic leukaemia (CMML) and acquired haemophilia A (AHA) are rare blood disorders. CMML is a clonal haematopoietic stem cell disorder, characterized by absolute monocytosis in the peripheral blood that persists for at least 3 months. According to the World Health Organization (WHO) classification, CMML is classified as a myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN). The incidence of CMML is lower than 10 cases per million people per year [1]. AHA is a bleeding disorder caused by autoantibodies against factor VIII (FVIII) with an estimated incidence of 1.48 cases per million people per year [2]. Although the underlying disorder cannot be identified in 50% of the patients [3], it is believed that AHA is associated with autoimmune disorders, malignancies, pregnancy, dermatological disorders and drug reactions. In this article, we describe a case of AHA in an old man with CMML; it is the fourth case of CMML associated with AHA to be reported in the literature, which makes the findings rare and valuable. Case ReportA 71-year-old Chinese male was admitted to the Department of Nephrology with a 3-day history of gross haematuria. In the course of the disease, he denied experiencing frequent, urgent micturition or urodynia. His medical history was unremarkable, except for hypertension that was controlled with plendil. He had neither a personal nor a family history of spontaneous bleeding. Physical examination detected multiple small ecchymoses on his left forearm. Moder-ate oedema was also found in both lower extremities. Laboratory examinations found white blood cell count (WBC, 79.48 9 10 9 L À1 ) and monocyte count (MO, 5.37 9 10 9 L À1 ) elevated, severe anaemia [haemoglobin (Hb), 53 g L À1 ] and thrombocytopenia [platelet count (PLT), 57 9 10 9 L À1 ]. Prolonged activated partial thromboplastin time (APTT) (118.6 s; normal range, 31-45 s) and deficiency in FVIII coagulant activity (FVIII:C) (6.70%; normal range, 50-150%) were also detected. Prothrombin time (PT), von Willebrand factor and other factors (II, V, VII, IX, X, XI and XII) were all normal. Factor VIII inhibitor was positive at a titre of 7.4 BU (normally it is negative). Urine examination found excessive amount of RBC. Rheumatoid factor and any autoantibodies such as anti-nuclear antibody, anti-dsDNA antibody, anti-Smith antibody, anti-cardiolipin antibody and lupus anticoagulant were negative. Bone marrow (BM) examination showed that the hypercellular marrow comprised 2% blasts with erythroid and megakaryocytic dysplasia. The karyotype was found to be normal (46,XY) by G-banding analysis, and the BCR/ ABL fusion gene was not detected by fluorescence in situ hybridization.The patient was diagnosed with CMML-1 with AHA based on the above findings, and transferred to our department for further treatment at the 7th hospital day. At that time, it revealed the presence of multiple large ecchymoses in the right thigh and lumbar back which caused by former BM aspiration. Treatment with hydroxycarbamide (HU) at a dose of 500 mg da...

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“…The reason for this may be due to the rare incidental rate of acquired haemophilia. Several cases of AHA complicated with CMML or MDS have been reported [11–16]. Including our case, four out of eight AHA cases went into remission after treatment; three of these cases were administered a hypomethylating agent (AZA or decitabine) for CMML (Table 3).…”

Section: Discussionmentioning

confidence: 84%

A Case of Acquired Haemophilia A in a Patient with Chronic Myelomonocytic Leukaemia

Araki

Ohata

Okamoto

et al. 2019

Case Reports in Hematology

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A 67-year-old male, with a known diagnosis of myelodysplastic syndromes with multilineage dysplasia (MDS-MLD) was admitted to our hospital with a primary complaint of subcutaneous bleeding in his left thigh. Laboratory data showed anaemia and prolongation of activated partial thromboplastin time (85.8 s, normal range 24–39 s) without thrombocytopenia. Coagulation factor VIII (FVIII) activity was less than 1% (normal range 60–150%), and a FVIII inhibitor was identified and quantified at 166 BU/mL to indicate a diagnosis of acquired haemophilia A (AHA). A recent, but sustained circulating monocytosis (>1 × 109/L) was observed, which combined with elevated numbers of neutrophil and monocytic cells in the marrow, suggested evolution of MDS-MLD to chronic myelomonocytic leukaemia (CMML), coinciding with AHA. Further analysis revealed a karyotype of 46, XY, i(14) (q10), which was the same abnormality previously identified in the patient. To treat bleeding caused by AHA, steroid and activated prothrombin complex concentrate were administered. Azacitidine (AZA) was used to treat CMML. During the clinical course, bleeding partially improved; however, subsequent acute myocardial infarction occurred on day 87. Worsening bone marrow failure was observed 4 months after the original admission, despite administration of AZA therapy, and the patient died due to bleeding from AHA. This case suggests that the evolution of MDS to CMML status can be associated with AHA conferring a bleeding tendency.

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Two cases of acquired haemophilia A associated with chronic myelomonocytic leukaemia

Cited by 6 publications

References 9 publications

The first case report of a patient with simultaneous haemophilia A and accelerated phase chronic myelogenous leukaemia

The first case report of a patient with simultaneous haemophilia A and accelerated phase chronic myelogenous leukaemia

Chronic myelomonocytic leukaemia associated with acquired haemophilia A: case report and literature review

A Case of Acquired Haemophilia A in a Patient with Chronic Myelomonocytic Leukaemia

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