Topic: 2007: HES possibly caused by Babesiosis species
CaliforniaLyme
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1: MedGenMed. 2007 Feb 27;9(1):38. Links
Are various Babesia species a missed cause for hypereosinophilia? A follow-up on the first reported case of imatinib mesylate for idiopathic hypereosinophilia.
Schaller JL, Burkland GA, Langhoff PJ.
INTRODUCTION: In 2001 we reported the first case of use of imatinib mesylate (Gleevec) for treatment of idiopathic hypereosinophilia syndrome (HES). These findings have been replicated in some patients with HES. After 1 year of taking imatinib, the patient stopped this medication, and during the last 5 years the patient has not experienced a relapse. He has, however, recently been diagnosed with babesiosis. This new diagnosis might relate to his HES.
METHODS: After 6 years we decided to follow up on this patient's treatment. We interviewed the patient, his son, his aunt, and 2 consulting physicians and also reviewed relevant laboratory results to determine whether his HES had returned and whether his residual morbidity had changed.
RESULTS: The patient has had no relapse of HES and his eosinophil counts have remained low-normal. He was recently diagnosed with babesiosis, and was prescribed atovaquone and azithromycin with a significant decrease in morbidity. His eosinophil cationic protein levels have also fallen to low-normal since starting atovaquone and azithromycin.
DISCUSSION: New Babesia species are emerging as human infections. Most do not have available antibody or polymerase chain reaction diagnostic testing at this time. Manual differential examinations are of variable utility due to low numbers of infected red blood cells, suboptimal technique, and limited experience. Therefore, a diagnosis might need to be empirical at times, and should be based on signs and symptoms.
CONCLUSION: The patient has not relapsed in the 5 years that he has not been taking imatinib. Babesiosis should be added to the many possible causes of HES.
It is unknown how often babesiosis causes HES as well as what percentage of HES patients have babesiosis.
PMID: 17435644 [PubMed - in process]
-------------------- There is no wealth but life. -John Ruskin
All truth goes through 3 stages: first it is ridiculed: then it is violently opposed: finally it is accepted as self evident. - Schopenhauer Posts: 5639 | From Aptos CA USA | Registered: Apr 2005
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What is HES?
Posts: 175 | From Pa | Registered: Aug 2006
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CaliforniaLyme
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HES is hypereosinophilia syndrome
QUOTE: History: Hypereosinophilia syndrome is a multisystem disease with symptoms related to eosinophil proteins and thrombotic phenomenon. Constitutional symptoms include fever, night sweats, anorexia, weight loss, fatigue, and nausea. Alcohol intolerance is occasionally noted.
Peripheral neuropathy (This may occur as symmetric or asymmetric sensory neuropathy, painful paresthesias, mixed sensory and motor neuropathy, mononeuritis multiplex, or radiculopathy.)
Author: Bruce M Rothschild, MD, Profess or of Medicine, The Northeastern Ohio Universities College of Medicine; Director, Arthritis Center of Northeast Ohio; Adjunct Professor, Department of Biomedical Engineering, University of Akron
Background: Hypereosinophilic syndrome varies from an asymptomatic phenomenon to a life-threatening multisystem disease. It is recognized on the basis of more than 1500 eosinophils per microliter (usually many more) for more than 6 months and multiorgan involvement in the absence of other causes of eosinophilia and in the absence of eosinophil blast cells in the marrow or blood.
Hypereosinophilic syndrome is very rare in children.
Pathophysiology: Hypereosinophilia appears to be caused by eosinophilopoietin cytokines, including interleukin 5 (IL-5), interleukin 3 (IL-3), and granulocyte/monocyte cell-stimulating factor; large numbers of circulating eosinophils result.
Toxicity is related to fibrosis, especially endomyocardial fibrosis, caused by eosinophil granules, including cationic granule proteins (eg, eosinophil-derived neurotoxin, eosinophil peroxidase, major basic protein, eosinophil cationic protein, transforming growth factor a and b), tumor necrosis factor a, interleukin 1a (IL-1a), macrophage inflammatory protein, interleukin 6 (IL-6), interleukin 8 (IL-8), IL-5, IL-3, and granulocyte/monocyte cell-stimulating factor.
Urokinase-induced plasminogen activation and factor XII-dependent reactions predispose the patient to thrombotic reactions.
Frequency:
In the US: Hypereosinophilia is rare, especially in children.
Internationally: Hypereosinophilia is rare, especially in children.
Mortality/Morbidity: Death generally results from primary heart damage or secondary endocarditis. Survival is prolonged if the sequelae of organ damage, especially cardiac organ damage, can be controlled. Mean survival is 9 months; the 3-year survival rate is reported to be 12%.
Poor prognostic indicators include the following:
Anemia
Thrombocytopenia
A WBC count higher than 100,000 cells per cubic millimeter
Abnormal circulating basophilic cells
Abnormal bone marrow
An elevated vitamin B-12 level
Abnormal leukocyte alkaline phosphatase levels Race: The prevalence low, with a distribution of cases, is as follows: 78% whites, 18% blacks, and 4% Asian Americans.
Sex: Hypereosinophilic syndrome has a 90% male predominance.
Age: Persons aged 5-80 years can have hypereosinophilic syndrome, which most commonly affects those persons aged 41-50 years. The disease is rare in children.
One report documents a case of eosinophilia (WBC, 80,000 per cubic millimeter with 63% eosinophils) in an infant born to a mother with hypereosinophilic syndrome. The child's eosinophil count returned to normal in 8 months.
History: Hypereosinophilia syndrome is a multisystem disease with symptoms related to eosinophil proteins and thrombotic phenomenon. Constitutional symptoms include fever, night sweats, anorexia, weight loss, fatigue, and nausea. Alcohol intolerance is occasionally noted.
Peripheral neuropathy (This may occur as symmetric or asymmetric sensory neuropathy, painful paresthesias, mixed sensory and motor neuropathy, mononeuritis multiplex, or radiculopathy.)
Hypereosinophilic syndrome is recognized on the basis of more than 1500 eosinophils per microliter, usually many more. The erythrocyte sedimentation rate (ESR) is usually elevated, but it can be normal. Leukocyte alkaline phosphatase levels may be high or low. Hypergammaglobulinemia in the form of extremely high immunoglobulin E (IgE) levels is noted in one third of patients. Immunoglobulin G (IgG), immunoglobulin M (IgM), and immunoglobulin A (IgA) levels are only rarely elevated. Rheumatoid factor is only rarely present. Coombs test results may be positive. Results of rapid plasma reagent (RPR) tests are rarely positive. Hematuria, proteinuria, and azotemia may be present. Synovial fluid eosinophilia is prominent. Chromosomal analysis rarely reveals abnormalities. When abnormal chromosomes are present, a workup for eosinophilic leukemia is recommended. (Eosinophilic leukemia is an unrelated disorder. The presence of chromosome abnormalities or eosinophil blast cells in the circulating blood or marrow may allow the leukemia to be recognized.) A laboratory workup is also indicated to rule out disorders that can mimic the hypereosinophilic syndrome (see Differentials). Imaging Studies:
Chest radiography may reveal nonspecific focal or diffuse, interstitial, or alveolar infiltrates. CT scanning may reveal pulmonary nodules with a halo of ground-glass attenuation, as well as nonspecific focal or diffuse, interstitial, or alveolar infiltrates. Ultrasonography of the affected liver may be normal. Technetium-99m sulfur colloid scanning may reveal nonhomogenous uptake in the presence of liver disease. Other Tests:
Electrocardiography may reveal atrial fibrillation, ST-segment depression, T-wave changes, conduction abnormalities, left atrial atrophy, or left ventricular hypertrophy. Electrocardiography reveals involvement in 90% of patients with hypereosinophilic syndrome. Procedures:
Bone marrow biopsy may reveal myelofibrosis. The presence of eosinophil blast cells suggests the alternative diagnosis of eosinophilic leukemia, an unrelated disorder. Histologic Findings: Activated eosinophil levels are noted in the blood and involved structures; this findings is associated with fibrosis. TREATMENT Section 6 of 10 Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Bibliography
Medical Care:
No therapy is indicated in absence of organ damage.
Angioedema and urticaria suggest a benign course.
Mucosal ulcers do not respond to corticosteroids.
Rapid intervention for cardiac disease is essential. Reducing the eosinophil load is the major goal of treatment. Aggressive control of eosinophilia seems important but hypothetical because all reports about treatment approaches are essentially anecdotal for this rare disorder. In the absence of organ disease, however, any indication for treatment is unclear except with respect to thrombosis risk. Therapy to prevent the risk of thrombosis may be reasonable for all patients. Surgical Care:
Cardiac surgery is indicated for annuloplasty, valve replacement, thrombectomy, and aortic prostheses. Because patients with mechanical valve replacements are especially prone to thrombosis, bioprostheses are recommended. Splenectomy may ameliorate platelet sequestration and is indicated for splenic infarction and pain due to splenic distention. Consultations: Obtaining subspecialty input from a rheumatologist and hematologist is essential.
MEDICATION Section 7 of 10 Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Bibliography
No therapy is indicated in the absence of organ damage. Treatment is directed at organ system involvement and at reducing the eosinophil load and perhaps the eosinophil effect.
-------------------- There is no wealth but life. -John Ruskin
All truth goes through 3 stages: first it is ridiculed: then it is violently opposed: finally it is accepted as self evident. - Schopenhauer Posts: 5639 | From Aptos CA USA | Registered: Apr 2005
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