|55 Kenosia Avenue
Danbury, CT 06810
Toll Free: 1.800.999.6673
The National Organization for Rare Disorders (NORD) web site, its databases, and the contents thereof are copyrighted by NORD. No part of the NORD web site, databases, or the contents may be copied in any way, including but not limited to the following: electronically downloading, storing in a retrieval system, or redistributing for any commercial purposes without the express written permission of NORD. Permission is hereby granted to print one hard copy of the information on an individual disease for your personal use, provided that such content is in no way modified, and the credit for the source (NORD) and NORD’s copyright notice are included on the printed copy. Any other electronic reproduction or other printed versions is strictly prohibited.
The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.
Copyright 1986, 1989, 1990, 1994, 2001, 2003, 2009
Babesiosis is a rare infectious disease caused by single-celled microorganisms (protozoa) belonging to the Babesia family. It is believed that the Babesia protozoa are usually carried and transmitted by ticks (vectors). Babesiosis occurs primarily in animals; however, in rare cases, babesiosis infection may occur in humans. Certain Babesia species are known to cause babesiosis infection in humans (e.g., Babesia microti), and the deer tick is a known vector. Human babesiosis infection may cause fever, chills, headache, nausea, vomiting, and/or muscle aches (myalgia). Symptoms may be mild in otherwise healthy people; in addition, some infected individuals may exhibit no symptoms (asymptomatic). However, a severe form of babesiosis, which may be life-threatening if untreated, may occur in individuals who have had their spleens removed (splenectomized) or who have an impaired immune system. A different form of babesiosis has been reported in Europe that is associated with a more severe expression of symptoms.
Most people with babesiosis do not experience any symptoms (asymptomatic) or experience only mild symptoms. However, in some cases, babesiosis can cause severe complications. These severe cases usually only occur in individuals greater than 50 years of age, individuals who have impaired immune systems (immunocompromised), or individuals who have had their spleen removed (splenectomized).
The symptoms associated with babesiosis usually develop approximately one to four weeks (incubation period) after exposure to the parasite. Symptoms vary greatly from case to case. The initial symptoms may include fever, a general feeling of ill health (malaise), fatigue, and loss of appetite. Additional early symptoms include joint pain (arthralgia), muscle pain (myalgia), chills, sweats, and headaches.
Affected individuals may also have additional symptoms including nausea, vomiting, and/or abdominal pain. In some cases, an abnormally large liver and or spleen (hepatosplenomegaly) may be present. Immune compromised individuals with severe babesiosis may have impaired kidney function and an abnormal yellow discoloration to their skin, mucous membranes, and whites of the eyes (jaundice).
Laboratory examination of blood samples from affected individuals may reveal abnormally low levels of red blood cells (hemolytic anemia) due to their destruction by the parasite. Additional laboratory findings may include abnormally low levels of platelets (thrombocytopenia) and white blood cells (leukopenia).
In 20-25 percent of human babesiosis cases, affected individuals also have Lyme disease. Individuals who have simultaneous infection with both diseases usually experience more severe expression of symptoms and a longer duration of those symptoms. Individuals with babesiosis may also have another infectious disease known as ehrlichiosis. In rare cases, an individual can be simultaneously affected by all three diseases.
In rare cases, this infection may be responsible for a respiratory condition known as adult respiratory distress syndrome (ARDS). (For more information on this disorder, choose "Adult Respiratory Distress" as your search term in the Rare Disease Database.)
Babesiosis is caused by single-celled microorganisms (protozoa) from the genus Babesia. These microorganisms are parasites that invade red blood cells (erythrocytes).
There are more than 100 species of Babesia. In most cases, the two species of Babesia that cause disease in humans (pathogenic) are Babesia microti and Babesia divergens. The species involved varies depending upon specific geographic location.
The major cause of babesiosis in the northeastern United States is infection by B. microti. In California and Washington a new Babesia parasite, called WA-1, is believed to be responsible for the disorder. In Europe, B. divergens and B. bovis are usually responsible for babesiosis.
Babesia protozoa such as B. microti are transmitted to humans through the bite of infected ticks. The ticks serve as "vectors," the term for any organism that is infected with and later transmits a particular disease agent (e.g., bacterium or virus) to another organism, which may then become infected. The deer tick (Ixodes dammini or scapularis) is the most common vector that transmits babesiosis.
In extremely rare cases, babesiosis may be transmitted following a blood transfusion with blood that is contaminated with the microorganism.
Babesiosis is a rare infectious disease that affects males and females in equal numbers. It can affect individuals of any age although it is more likely to occur in people over 50 years of age, people who have weakened immune systems, and people who have had their spleens removed.
In the United States, most cases are confined to the northeastern coast in the states of New York, Massachusetts, and Connecticut. The infected tick is present in greater numbers in these areas (endemic). Islands off the northeastern coast including Long Island, Martha's Vineyard, Nantucket, and Block Island are also areas where the tick may be found. Cases of babesiosis have also been reported in Washington, California, Georgia, New Jersey and Wisconsin. A more serious form of babesiosis occurs in Europe.
Approximately 200 cases of babesiosis were reported in the United States during the 1980s. More than 450 cases have been reported in the medical literature. However, because some affected individuals may develop few associated symptoms and findings, the disorder may often remain unrecognized and may therefore be underdiagnosed, making it difficult to determine the true frequency of babesiosis in the general population. The disease has been diagnosed with greater frequency during the 1990s.
Symptoms of the following disorders can be similar to those of babesiosis. Comparisons may be useful for a differential diagnosis:
Malaria is an infectious disease that is caused by single-celled microorganisms (protozoa) of the Plasmodium genus and spread by the bite of an infected mosquito (Anopheles). Major symptoms may include fever, chills, listlessness, loss of appetite, headaches, muscle aches, and other flu-like symptoms. Additional symptoms may include muscle spasms and rigidity, profuse sweating, cold sores, abnormally fast heartbeat, and difficulty breathing. The severity of the symptoms varies greatly from case to case. Low levels of circulating red blood cells (anemia), weight loss, and a mild yellowish discoloration of the skin, mucous membranes, and whites of the eyes (jaundice) may also occur. Four different species of the Plasmodium genus may cause malaria. (For more information on this disorder, choose "Malaria" as your search term in the Rare Disease Database.)
Lyme disease is an infectious disease caused by the spirochete bacterium Borrelia burgdorferi. The bacterium is carried and transmitted by deer ticks (Ixodes dammini). In most cases, Lyme disease is first characterized by the appearance of a red skin lesion (erythema chronicum migrans), which begins as a small elevated round spot (papule) that expands to at least five centimeters in diameter. Symptoms may then progress to include low-grade fever, chills, muscle aches (myalgia), headache, a general feeling of weakness and fatigue (malaise), and/or pain and stiffness of the large joints (infectious arthritis), especially in the knees. Such symptoms may tend to occur in recurrent cycles. In severe cases, heart muscle (myocardial) and/or neurological abnormalities may occur. Most cases of Lyme disease occur in the northeastern United States. However, cases have occurred in other areas of the United States as well as other countries including China, Japan, Australia, and several countries in Europe. (For more information on this disease, choose "Lyme" as your search term in the Rare Disease Database.)
Toxoplasmosis is an infectious disorder that is caused by a single-celled parasite known as Toxoplasma gondii that often infects cats. This infection is found worldwide and may be acquired or transmitted to a fetus from an infected mother. Individuals may also develop toxoplasmosis by eating partially cooked meat especially pork. Accidental swallowing of Toxoplasma eggs also results in development of toxoplasmosis. This may occur by touching one's mouth after gardening near dirt infected with the eggs or cleaning the litter of an infected cat. Symptoms may include fatigue, rash, high fever, chills, and inflammation of the liver (hepatitis). Lesions may also occur in the lungs, heart, skin, muscle, and/or brain. (For more information on this disorder, choose "Toxoplasmosis" as your search term in the Rare Disease Database.)
Human monocytic ehrlichiosis (HME), a rare infectious disease, is caused by a bacterium from the "Ehrlichia" family known as Ehrlichia chaffeensis. The invading bacteria spread through blood and lymphatic vessels and invade certain cells that play an essential role in the body's immune system (monocytes and macrophages). In individuals with HME, the onset of symptoms usually occurs about three weeks after an individual has been bitten by a tick carrying the E. chaffeensis bacterium. Symptoms may initially include fever, chills, headaches, muscle pain (myalgia), and a general feeling of weakness and fatigue (malaise). Symptoms may then progress to include nausea, vomiting, loss of appetite (anorexia), and/or weight loss. Some affected individuals may also experience coughing, diarrhea, sore throat (pharyngitis), pain in the abdominal area, and/or confusion. Most cases have occurred in the mid-Atlantic and southeastern states in the United States. (For more information on this disease, choose "Human Monocytic Ehrlichiosis" as your search term in the Rare Disease Database.)
Human granulocytic ehrlichiosis (HGE), a rare infectious disease, is caused by a bacterium from the "Ehrlichia" family that has not yet been named. The bacterium, which is carried and transmitted by ticks (vectors), invades certain granular white blood cells (neutrophils) that play a role in engulfing bacteria, removing them from the blood, and destroying them (phagocytosis). In individuals with HGE, the onset of symptoms usually occurs approximately one week after an individual has been bitten by a tick carrying the Ehrlichia bacterium. In almost all cases, symptoms include fever, chills, muscle pain (myalgia), a general feeling of weakness and fatigue (malaise), and/or headaches. Some affected individuals may also experience coughing, nausea, vomiting, and/or confusion. Most cases have affected individuals in the Northeastern and Midwestern United States. (For more information on this disorder, choose "Human Granulocytic Ehrlichiosis" as your search term in the Rare Disease Database.)
The diagnosis of babesiosis is made based upon a thorough clinical evaluation, a detailed patient history (e.g., recent tick bite), characteristic findings, and specialized tests such as the examination of blood smears that screen for the parasite inside red blood cells (erythrocytes). The diagnosis may also be confirmed by antibody testing (indirect immunofluorescent assay).
In most healthy people, babesiosis usually resolves spontaneously and causes few or no symptoms. People with an impaired immune system may require treatment with drugs such as clindamycin, quinine, and/or other antiparasitic or antibiotic drugs. Clindamycin and quinine are the drugs most commonly used to treat individuals with severe symptoms of babesiosis.
Treatment with two different drugs, atovaquone and azithromycin, has been used in cases where clindamycin and quinine were ineffective. Individuals who have had their spleens removed who have severe cases of babesiosis may be treated with blood transfusions.
Individuals who will be exposed to areas with high numbers of tick vectors for the Babesia parasites (e.g., fields, wooded or marsh areas, etc.) should consider taking certain steps to prevent infection. Such steps should include wearing long-sleeved shirts, long pants, and hats; wearing light-colored clothing to make ticks more visible; using appropriate tick repellents; and carefully checking clothing and skin (particularly the scalp and the back of the neck) after being in such locations.
Research on tropical diseases is ongoing. For more information about these disorders, contact the World Health Organization (WHO) listed in the Resources below.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
Bennett JC, Plum F, eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996:1915-16.
Fauci AS, et al., eds. Harrison's Principles of Internal Medicine, 14th Ed. New York, NY: McGraw-Hill, Inc; 1998:1188-89.
Peter, G, et al., eds. Report of the committee on infectious diseases, 21st ed. Am Acad of Peds;1988:131.
Krause PJ. Babesiosis diagnosis and treatment. Vector Borne Zoonotic Dis. 2003;3:45-51.
Herwaldt BL, et al. Endemic babesiosis in another eastern state: New Jersey. Emerg Infec. Dis. 2003;9:184-8.
Gelfand JA, Callahan MV. Babesiosis: an update on epidemiology and treatment. Curr Infect Dis Rep. 2003;5:53-8.
Weiss LM. Babesiosis in humans: a treatment review. Expert Opin Pharmacother. 2002;3:1109-15.
Kjemtrup AM, et al. Human babesiosis: an emerging tick-borne disease. Int J Parasitol. 2000;30:1323-37.
Krause PJ, et al. Atovaquone and axithromycin for the treatment of babesiosis. N Engl J Med. 2000;343:1454-58.
Fell E. An update on Lyme disease and other tick-borne diseases. Nurse Pract. 2000;10:38-40, 43-4, 47-48.
Perdrizet GA, et al. Babeiosis in a renal transplant recipient acquired through blood transfusion. Transplantation. 2000;70:205-08.
Homer MJ, et al. Babesiosis. Clin Microbiol Rev. 2000;13:451-69.
Linden JV, et al. Transfusion-associated transmission of babesiosis in New York State. Transfusion. 2000;40:285-89.
Meldrum SC. Human babesiosis in New York State: an epidemiological description of 136 cases. Clin Infect Dis. 1992;15:1019-23.
Carr JM, et al. Babesiosis, diagnostic pitfalls. Am J Clin Pathol. 1991;95:774-77.
Doan-Wiggins L. Tick-borne diseases. Emerg Med Clin North Am. 1991;9:303-25.
Krause PJ, et al. Babesiosis: an underdiagnosed disease of children. Pediatrics. 1992;89:1045-48.
Mintz ED. Transfusion-transmitted babesiosis: a case report from a new endemic area. Transfusion. 1991;31:365-68.
Mathewson HO, et al. Self-limited babesiosis in a splenectomized child. Ped Infect Dis Jour. 1990;3:66-67.
Jacobs R. Tick exposure and related infections. Ped Infect Dis Jour. 1988;7:342-46.
Report last updated: 2009/04/08 00:00:00 GMT+0