|LETTER TO EDITOR
|Year : 2019 | Volume
| Issue : 1 | Page : 22-23
Idiopathic hypereosinophilic syndrome and disseminated intravascular coagulation
Mansoor C Abdulla
Department of General Medicine, MES Medical College, Perinthalmanna, Kerala, India
|Date of Submission||21-Feb-2019|
|Date of Acceptance||18-Mar-2019|
|Date of Web Publication||28-Mar-2019|
Dr. Mansoor C Abdulla
Department of General Medicine, MES Medical College, Perinthalmanna, Kerala
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Abdulla MC. Idiopathic hypereosinophilic syndrome and disseminated intravascular coagulation. Cancer Transl Med 2019;5:22-3
Hypereosinophilic syndromes (HESs) are conditions characterized by persistent eosinophilia of 1.5 × 109/L or higher and manifestations of end-organ involvement or dysfunction attributable to the eosinophilia. Immunological, infectious, and malignant disorders can be associated with HESs but can be even idiopathic. Disseminated intravascular coagulation (DIC) associated with HES is rare. We report a case of HES complicated by DIC which improved with treatment.
A 45-year-old woman was admitted with generalized itching for 6 months and breathlessness for 1 week. She had a history of hemorrhoids for the past 6 years. She denied a history of weight loss, had no sick contacts, and had no history of addictions. Examination showed multiple purpuric spots over both hand and foot and a Grade 3/5 pansystolic murmur over the tricuspid area. There were no other bleeding manifestations. Hemoglobin was 10.0 g/dl, total leukocyte count – 19,700/μl (neutrophil – 39%, lymphocytes – 23%, eosinophils – 33%, and monocytes – 5%), platelet count – 57,000/L, and erythrocyte sedimentation rate – 8 mm in 1 h. Peripheral smear showed hemolysis, severe eosinophilia, and moderate thrombocytopenia [Figure 1]a. Direct and indirect Coombs tests were negative. Renal function tests, blood sugar, serum electrolytes, urinalysis, and chest radiograph were all normal. Liver function tests showed indirect hyperbilirubinemia. Prothrombin time and activated partial thromboplastin time (aPTT) were both prolonged. Serum lactate dehydrogenase, D-dimer, and fibrin degradation product level were high. HIV, HbsAg, and HCV serologies were negative. Bone marrow examination showed trilineage maturation and myeloid hyperplasia with increase in eosinophils and its precursors [Figure 1]b. Workup for hypereosinophilia including stool examination for parasites, antinuclear antibody, and antineutrophil cytoplasmic antibody was negative. Cytogenetic studies for detecting FIP1L1-PDGFRA rearrangement, CHIC2 deletion, FGFR1 rearrangement, and breakpoint cluster region-Abelson murine leukemia were negative. Ultrasonography abdomen showed a hypoechoic lesion in the left lobe of the liver (48 mm × 38 mm). Contrast-enhanced computed tomography of the abdomen showed an exophytic, hypodense lesion measuring 8.1 cm × 7 × cm 4.2 cm in the segment IV of the liver, and on postcontrast imaging, the lesion showed gradual centripetal filling in the arterial, portal, and delayed phases, which was suggestive of hepatic hemangioma. Echocardiogram showed thickened posterior mitral valve leaflet, moderate tricuspid and mitral regurgitation, severe pulmonary arterial hypertension, left ventricular apical and lateral wall fibrosis and right ventricular apical fibrosis, and dysfunction [Figure 2]. Contrast-enhanced computed tomography thorax was normal. A diagnosis of DIC complicating idiopathic HES with cardiac involvement was made. She was treated with intravenous methylprednisolone 1 g daily for 3 days, which was followed by oral prednisolone (1 mg/kg body weight). Her breathlessness improved following the treatment, but the eosinophilia was persisting and thus was started on hydroxyurea 1 g daily with which the eosinophil count became normal.
|Figure 1: Photomicrograph of peripheral smear showing hemolysis, severe eosinophilia, and moderate thrombocytopenia (a). Bone marrow biopsy showing myeloid hyperplasia with increase in eosinophils and its precursors on hematoxylin and eosin stain (b)|
Click here to view
|Figure 2: Echocardiogram showing thickened posterior mitral valve leaflet, moderate tricuspid and mitral regurgitation (a) and left ventricular apical and lateral wall fibrosis and right ventricular apical fibrosis (b and c)|
Click here to view
The etiology of the eosinophilia in HESs can be primary (myeloid), secondary (lymphocyte driven), or unknown. HES can be classified into six clinical variants: myeloproliferative HE/HES (M-HE/M-HES), lymphocytic variant HE/HES (L-HE/L-HES), overlap HES, associated HE/HES, familial HE/HES, and idiopathic HES. Fifty percent of HES is idiopathic even after extensive evaluation.
Thromboembolism is a common complication of HES (seen in 25%) and has a high mortality (5%–10% die as a result). The actual mechanisms responsible for HES-derived coagulopathy are not well elucidated. The four main granule proteins released by eosinophils including major basic protein, eosinophil-derived neurotoxin, eosinophil cationic protein, and eosinophil peroxidase are thought to be responsible for the hypercoagulable state. Manifestations related to DIC result from hypercoagulation and hyperfibrinolysis. When both factors are weak, there are no clinical symptoms, although abnormalities in clinical laboratory tests can be present; this type of DIC is called the nonsymptomatic type of DIC or pre-DIC which was present in our case.,
DIC as a complication of HES was reported previously.,,, Our patient only had purpuric spots over the extremities and laboratory evidence for DIC such as thrombocytopenia, microangiopathic hemolysis, prolonged prothrombin time and aPTT, and high D-dimer and fibrin degradation product levels. Most of the previously described cases presented with significant thrombotic manifestations secondary to DIC. The patient showed an excellent response to treatment. Early recognition of rare and fatal complications in patients with HES is crucial for precise management.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Klion AD. How I treat hypereosinophilic syndromes. Blood
2015; 126(9): 1069–77.
Wang JG, Mahmud SA, Thompson JA, Geng JG, Key NS, Slungaard A. The principal eosinophil peroxidase product, HOSCN, is a uniquely potent phagocyte oxidant inducer of endothelial cell tissue factor activity: a potential mechanism for thrombosis in eosinophilic inflammatory states. Blood
2006; 107(2): 558–65.
Taylor FB Jr., Toh CH, Hoots WK, Wada H, Levi M; Scientific Subcommittee on Disseminated Intravascular Coagulation (DIC) of the International Society on Thrombosis and Haemostasis (ISTH). Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost
2001; 86(5): 1327–30.
Wada H, Matsumoto T, Yamashita Y. Diagnosis and treatment of disseminated intravascular coagulation (DIC) according to four DIC guidelines. J Intensive Care
2014; 2(1): 15.
Yeung TF, Lau SW, Wong K. An unusual case of hypereosinophilic syndrome and disseminated intravascular coagulation. Chin Med J (Engl)
2005; 118(18): 1582–4.
Miyagi J, Ichimiya M, Ozaki K, Goto T, Fujino O, Nagata J, Hiasa Y. Hypereosinophilic syndrome complicated by disseminated intravascular coagulation (DIC), deep venous thrombosis and pulmonary embolism. Nihon Naika Gakkai Zasshi
2004; 93(2): 364–6.
Fukuta A, Hara T, Tsurumi H, Moriwaki H. Hypereosinophilic syndrome with DIC treated successfully with a combination of high-dose methylprednisolone and cyclosporin A. Rinsho Ketsueki
2001; 42(11): 1145–7.
Park SM, Park JW, Kim SM, Koo EH, Lee JY, Lee CS, Choi DC, Lee BJ. A case of hypereosinophilic syndrome presenting with multiorgan infarctions associated with disseminated intravascular coagulation. Allergy Asthma Immunol Res
2012; 4(3): 161–4.
[Figure 1], [Figure 2]