Aplastic anemia is a condition where bone marrow does not produce sufficient new cells to replenish blood cells. The condition, per its name, involves both aplasia and anemia. Typically, anemia refers to low red blood cell counts, but aplastic anemia patients have lower counts of all three blood cell types: red blood cells, white blood cells, and platelets, termed pancytopenia.

Signs and symptoms

Anemia with malaise, pallor and associated symptoms such as palpitations

Thrombocytopenia (low platelet counts), leading to increased risk of hemorrhage, bruising and petechiae

Leukopenia (low white blood cell count), leading to increased risk of infection

Reticulocytopenia (low reticulocyte counts)

Causes

In many cases, the etiology is considered to be idiopathic (cannot be determined), but one known cause is an autoimmune disorder in which white blood cells attack the bone marrow.

Aplastic anemia is also sometimes associated with exposure to toxins such as benzene, or with the use of certain drugs, including chloramphenicol, carbamazepine, felbamate, phenytoin, quinine, and phenylbutazone. Many drugs are associated with aplasia mainly according to case reports but at a very low probability. As an example, chloramphenicol treatment is followed by aplasia in less than 1 in 40,000 treatment courses, and carbamazepine aplasia is even more rare.

Exposure to ionizing radiation from radioactive materials or radiation-producing devices is also associated with the development of aplastic anemia.

Aplastic anemia is present in up to 2% of patients with acute viral hepatitis.

In some animals aplastic anemia may have other causes. For example, in the ferret (Mustela putorius furo) aplastic anemia is caused by estrogen toxicity. This is because female ferrets are induced ovulators, so mating is required to bring the female out of heat. Intact females, if not mated, will remain in heat, and after some time the high levels of estrogen will cause the bone marrow to stop producing red blood cells.

Diagnosis

The condition needs to be differentiated from pure red cell aplasia. In aplastic anemia the patient has pancytopenia (i.e., anemia, neutropenia and thrombocytopenia) resulting in decrease of all formed elements. In contrast, pure red cell aplasia is characterized by reduction in red cells only. The diagnosis can only be confirmed on bone marrow examination. Before this procedure is undertaken, a patient will generally have had other blood tests to find diagnostic clues, including a complete blood count (CBC), renal function and electrolytes, liver enzymes, thyroid function tests, vitamin B₁₂ and folic acid levels.

Following tests aid in determining differential diagnosis for aplastic anemia: # Bone marrow aspirate and biopsy: to rule out other causes of pancytopenia (i.e. neoplastic infiltration or significant myelofibrosis). # History of iatrogenic exposure to cytotoxic chemotherapy: can cause transient bone marrow suppression # X-rays, computed tomography (CT) scans, or ultrasound imaging tests: enlarged lymph nodes (sign of lymphoma), kidneys and bones in arms and hands (abnormal in Fanconi anemia) # Chest X-ray: infections # Liver tests: liver diseases # Viral studies: viral infections # Vitamin B₁₂ and folate levels: vitamin deficiency # Blood tests for paroxysmal nocturnal hemoglobinuria # Test for antibodies: immune competency

Treatment

Treating immune-mediated aplastic anemia involves suppression of the immune system, an effect achieved by daily medicine intake, or, in more severe cases, a bone marrow transplant, a potential cure. The transplanted bone marrow replaces the failing bone marrow cells with new ones from a matching donor. The multipotent stem cells in the bone marrow reconstitute all three blood cell lines, giving the patient a new immune system, red blood cells, and platelets. However, besides the risk of graft failure, there is also a risk that the newly created white blood cells may attack the rest of the body ("graft-versus-host disease").

Medical therapy of aplastic anemia often includes a short course of anti-thymocyte globulin (ATG) or anti-lymphocyte globulin (ALG) and several months of treatment with ciclosporin to modulate the immune system. Mild chemotherapy with agents such as cyclophosphamide and vincristine may also be effective. Antibody therapy, such as ATG, targets T-cells, which are believed to attack the bone marrow. Steroids are generally ineffective, though are often used to combat serum sickness caused by ATG use.

One prospective study involving cyclophosphamide was terminated early due to a high incidence of mortality, due to severe infections as a result of prolonged neutropenia.

In the past, before the above treatments became available, patients with low leukocyte counts were often confined to a sterile room or bubble (to reduce risk of infections), as in the famed case of Ted DeVita.

Follow-up

Regular full blood counts are required to determine whether the patient is still in a state of remission.

10-33% of all patients develop the rare disease paroxysmal nocturnal hemoglobinuria (PNH, anemia with thrombopenia and/or thrombosis), which has been explained as an escape mechanism by the bone marrow against destruction by the immune system. Flow cytometry testing is performed regularly in people with previous aplastic anemia to monitor for the development of PNH.

Prognosis

Untreated aplastic anemia is an illness that leads to rapid death, typically within six months. If the disease is diagnosed correctly and initial treatment is begun promptly, then the survival rate for the next five to ten years is substantially improved, and many patients live well beyond that length of time.

Occasionally, milder cases of the disease resolve on their own. Relapses of previously controlled disease are, however, much more common. Relapse following ATG/ciclosporin use can sometimes be treated with a repeated course of therapy.

Well-matched bone marrow transplants from siblings have been successful in young, otherwise healthy people, with a long-term survival rate of 80%-90%. Most successful BMT recipients eventually reach a point where they consider themselves cured for all practical purposes, although they need to be compliant with follow-up care permanently.

Older people (who are generally too frail to undergo bone marrow transplants) and people who are unable to find a good bone marrow match, who undergo immune suppression have five year survival rates of up to 75%.

See also

Fanconi anemia

Acquired pure red cell aplasia

References

External links

Aplastic Anemia & MDS International Foundation

Mayo Clinic

University of Texas

-- Idiopathic aplastic anemia -- Secondary aplastic anemia

The Aplastic Anaemia Trust

Shannon's Trust, support for people with aplastic anemia

Category:Blood disorders Category:Autoimmune diseases Category:Anemias Category:Hematopathology

Coordinates	33°55′31″N18°25′26″N
Name	Rajesh Shah
Image size	200px
Birth date	1963
Birth place	India
Occupation	Homeopath
Website	www.rajeshshah.com

Rajesh Shah is a practicing in homeopath in Mumbai, India. He graduated from CMPH Medical College, Mumbai, in 1984 and is mentioned in Limca Book of Records, where he holds the world record for having patients in the most countries.

He has been known as a promoter of homeopathy. Using the internet as a medium, he has launched over twenty web sites which talk about the role, scope and limitation of homeopathic treatment for various diseases. He has made critical remarks towards some homeopathic approaches centered around the prescribing on the basis of imaginary, whimsical concepts of dreams, grand generalisaton, pseudoclassical homoeopathy.

External links

Dr. Rajesh Shah's website

Sify.com: An interview with Dr. Rajesh Shah

An interview with Dr Rajesh Shah published on rediff.com site, July 15, 2002

Press reports - The Times Of India, Hindustan Times, New Delhi, Indian Express, etc.

FAQ on homeopathy. An interview with Dr Rajesh Shah in Mumbai Mirror, 2009.

References

Category:Homeopaths

:This article is about the Quaker philanthropist. For the institutions named after him, see Johns Hopkins University and Johns Hopkins Hospital.

Coordinates	33°55′31″N18°25′26″N
Name	Johns Hopkins
Birth date	May 19, 1795
Birth place	Anne Arundel County, Maryland, U.S.
Death date	December 24, 1873
Death place	Baltimore, MD, U.S.
Occupation	Entrepreneur, Investor, Philanthropist, Abolitionist
Religion	Society of Friends (Quakerism)

Johns Hopkins (May 19, 1795 – December 24, 1873) was a wealthy entrepreneur, philanthropist, and abolitionist of 19th century Baltimore, now most noted for his philanthropic creation of the institutions that bear his name, namely the Johns Hopkins Hospital, and the Johns Hopkins University and its associated divisions, in particular the schools of nursing, medicine, and public health. A biography entitled Johns Hopkins: A Silhouette written by his cousin, Helen Hopkins Thom, was published in 1929 by the Johns Hopkins University Press.

Early life

On May 19, 1795, Johns Hopkins was born on Whitehall, a 500-acre (two km²) tobacco plantation with approximately 500 slaves located in Anne Arundel County, Maryland. which meant that the formal education of Johns, then 12, had to be interrupted in order to help out on the plantation. Moreover, his help was needed because he was the second oldest of eleven children and, as their local Friends society had decreed, the family freed only the able-bodied slaves, and had to provide for the less able-bodied slaves, who would remain at the plantation and provide labor as they were able. In 1812, at the age of 17, Hopkins left the plantation and went to Baltimore to work in the wholesale grocery business of his uncle, Gerard Hopkins. While living with his uncle's family, Johns and his cousin, Elizabeth, fell in love, but the taboo against the marriage of first cousins was especially strong among Quakers. Neither Johns nor Elizabeth ever married. Still, just as he would continue to provide for his extended family throughout his life and posthumously through his will, Hopkins bequeathed a home for Elizabeth, where she lived until her death in 1889.

Whitehall Plantation is now known as the Walden Community (Crofton, Maryland), however the house, since restored and modified, is located on Johns Hopkins Road, adjacent to Reidel Road. The heavily landscaped house is surrounded by Walden Golf Course, and contains a historic marker.

Philanthropy

Living his entire adult life in Baltimore, Hopkins made many friends among the city's social elite, many of them Quakers. One of these friends was George Peabody, who was also born in 1795, and who in 1857 founded the Peabody Institute in Baltimore. Other examples of public giving were evident in the city, as public buildings housing free libraries, schools and foundations sprang up along the city's widening streets. On the advice of Peabody, some believe, Hopkins determined to use his great wealth for the public good.

The Civil War had taken its toll on Baltimore, however, as did the yellow fever and cholera epidemics that repeatedly ravaged the nation's cities, killing 853 in Baltimore in the summer of 1832 alone. Hopkins was keenly aware of the city's need for medical facilities, particularly in light of the medical advances made during the war, and in 1870 he made a will setting aside seven million dollars - mostly in B&O; stock - for the incorporation of a free hospital and affiliated medical and nurse's training colleges, as well as an orphanage for colored children and a university. Many board members were on both boards. The hospital and orphan asylum would each be overseen by the 12-member hospital board of trustees, and the university by the 12-member university board of trustees. Johns Hopkins' bequest was used to found posthumously the Johns Hopkins Colored Children Orphan Asylum first as he requested, in 1875; the Johns Hopkins University in 1876; the Johns Hopkins Press, the longest continuously operating academic press in America, in 1878; the Johns Hopkins Hospital and the Johns Hopkins School of Nursing in 1889; the Johns Hopkins University School of Medicine 1893; and the Johns Hopkins School of Hygiene and Public Health in 1916.

Johns Hopkins' views on his bequests, and on the duties and responsibilities of the two board of trustees, especially the hospital board of trustees led by his friend and fellow Quaker Francis King, were formally stated primarily in four documents, the incorporation papers filed in 1867, his instruction letter to the hospital trustees dated March 12 1873, his will, which was quoted from extensively in his Baltimore Sun obituary, and in his will's two codicils, one dated 1870 and the other dated 1873.

In these documents, Hopkins also made provisions for scholarships to be provided for poor youths in the states where Johns Hopkins had made his wealth, as well as assistance to orphanages other than the one for African American children, to members of his family, to those he employed, black and white, his cousin Elizabeth, and, again, to other institutions for the care and education of youths regardless of color and the care of the elderly, and the ill, including the mentally ill, and convalescents.

John Rudolph Niernsee, one of most famous architects of the time, designed the orphan asylum and helped to design the Johns Hopkins Hospital. The original site for the Johns Hopkins University had been chosen personally by Hopkins. According to his will, it was to be located at his summer estate, Clifton. However, a decision was made not to found the university there. The property, now owned by the city of Baltimore, is the site of a golf course and a park named Clifton Park. While the Johns Hopkins Colored Children Orphan Asylum was founded by the hospital trustees, the other institutions that carry the name of "Johns Hopkins" were founded under the administration of the first president of the Johns Hopkins University and Johns Hopkins Hospital, Daniel Coit Gilman and his successors.

Colored Children Orphan Asylum

As per Johns Hopkins' instruction letter, the Johns Hopkins Colored Children Orphan Asylum (JHCCOA) was founded first, in 1875, a year before Gilman's inauguration, now the founding date of the university. The construction of the asylum, including its educational and living facilities, was praised by The Nation and the Baltimore American, the latter stating that the orphan asylum was a place where "nothing was wanting that could benefit science and humanity". As was done for other Johns Hopkins Institutions, it was planned after visits and correspondence with similar institutions in Europe and America.

The Johns Hopkins Orphan Asylum opened with 24 boys and girls. Under Gilman and his successors, this orphanage was later changed to serve as an orphanage and training school for black female orphans principally as domestic workers, and next as an "orthopedic convalescent" home and school for "colored crippled" children and orphans. The asylum was eventually closed in 1924 nearly fifty years after it opened, and was never reopened.

Hospital, University, Press, and Schools of Nursing and Medicine

As per Hopkins' March 1873 Instruction Letter, the School of Nursing was founded alongside the Hospital in 1889 by the hospital board of trustees in consultation with Florence Nightingale. Both the nursing school and the hospital were founded over a decade after the founding of the orphan asylum in 1875 and the university in 1876. Hopkins' instruction letter explicitly stated his vision for the hospital; first, to provide assistance to the poor of "all races", no matter the indigent patient's "age, sex or color"; second, that wealthier patients would pay for services and thereby subsidize the care provided to the indigent; third, that the hospital would be the administrative unit for the orphan asylum for African American children which was to receive $25,000 in annual support out of the hospital's half of the endowment; and fourth, that the hospital and orphan asylum should serve 400 patients and 400 children respectively, fifth, that the hospital should be part of the university, and, sixth, that religion but not sectarianism should be an influence in the hospital.

By the end of Gilman's presidency, Johns Hopkins University, Johns Hopkins Press, Johns Hopkins Hospital and Johns Hopkins School of Nursing, and Johns Hopkins School of Medicine, and Johns Hopkins Hospital Colored Children Orphan Asylum had been founded, the latter by the trustees, and the others in the order listed under the Gilman administration. "Sex" and "color" were major issues in the early history of the Johns Hopkins Institutions. The founding of the School of Nursing is usually linked to Johns Hopkins' statements in his March 1873 instruction letter to the trustees that "I desire you to establish, in connection with the hospital, a training school for female nurses. This provision will secure the services of women competent to care for those sick in the hospital wards, and will enable you to benefit the whole community by supplying it with a class of trained and experienced nurses".

Women's most well known success after the founding of the nursing school was their requirement that they be allowed to attend Johns Hopkins medical institutions after they provided funds that made possible the opening of the School of Medicine in 1893. Five African American women were among the first women enrolled in the Johns Hopkins University's undergraduate school in 1970. Kelly Miller and Frederick Scott were the first persons of African descent to attend the Johns Hopkins University's graduate and undergraduate schools, respectively. Frederick Scott was the first graduate of African descent from Johns Hopkins University, and he, Robert Gamble, and Kenyan-born James Nabwangu were the first graduates of African descent from Johns Hopkins University's undergraduate school, and Johns Hopkins' medical schools respectively. Those employees holding jobs in the service sector are those of African descent who have the longest and most continuous history at the Johns Hopkins Institutions.

Legacy

)]] Following Hopkins' death, the Baltimore Sun wrote a lengthy obituary which closed thus: "In the death of Johns Hopkins a career has been closed which affords a rare example of successful energy in individual accumulations, and of practical beneficence in devoting the gains thus acquired to the public." His contribution to the university that has become his greatest legacy was, by all accounts, the largest philanthropic bequest ever made to an American educational institution.

Johns Hopkins' Quaker faith and his early life experiences, in particular the 1807 emancipation, had a lasting influence through out his life and his posthumous legacy as a businessman, railroad man, banker, investor, ship owner, philanthropist and a founder of several Institutions. From very early on, Johns Hopkins had looked upon his wealth as a trust to benefit future generations. He is said to have told his gardener that, "like the man in the parable, I have had many talents given to me and I feel they are in trust. I shall not bury them but give them to the lads who long for a wider education"; his philosophy quietly anticipated Andrew Carnegie's much publicized Gospel of Wealth by more than 25 years.

His philanthropy, banking and other business practices were founded neither on slavery nor on the separate but unequal racism of the post Civil War years of his life. In this vein, integral parts of his legacy, as an emancipator, a founder of an orphan asylum for African-American youths, a staunch advocate of abolitionism and of quality care not just for those physically ill, but also for the elderly, the poor, no matter their age, sex, or color, and the mentally ill, have by and large been overlooked, even in the institutions that carry his name.

In 1973 Johns Hopkins was cited prominently in the Pulitzer Prize winning book The Americans: The Democratic Experience by Daniel Boorstin, former head of the Library of Congress. From November 14, 1975 to September 6, 1976 Hopkins portrait was displayed at the National Portrait Gallery in an exhibit on the democratization of America based on Boorstin's book. In 1989, the United States Postal Service issued a $1 postage stamp in Johns Hopkins' honor, as part of the Great Americans series.

References

External links

Genealogical Records on Marylanders

Thom and Jacob discuss his love for his cousin and Quaker traditions

In his 1887 memoir, Baltimore and the Nineteenth of April, 1861: A Study of the War, George William Brown city Johns Hopkins as a wealthy Union man in Baltimore, a city with strong Confederate and Southern leanings

In The Chronicles of Baltimore: Being a Complete History of "Baltimore Town" and Baltimore City from the Earliest Period to the Present Time published in 1874, John Thomas Scharf cited the 1873 instruction letter to the hospital trustees and a city council resolution thanking Johns Hopkins for his philanthropy. Thom's biography and New York and Maryland newspapers were sources that published parts or all of this letter

The Institutional Records of The Johns Hopkins Hospital Colored Orphan Asylum

Abstract Johns Hopkins Dream for a Model of its Kind: The JHH Colored Orphans Asylum", 2000 Conference International Society for the History of Medicine BY Dr. P. Reynolds

Grave site of Johns Hopkins

Graveside ceremony for Johns Hopkins

Baltimore and the Nineteenth of April, 1861: A Study of the War, the memoir of George William Brown then the mayor of Baltimore city, later a member of the university board of trustees of the Johns Hopkins University

The Chronicles of Baltimore: Being a Complete History of "Baltimore Town" and Baltimore City from the Earliest Period to the Present Time published in 1874 by John Thomas Scharf

"If He Could See Us Now: Mr. Johns Hopkins' Legacy Strong University, Hospital Benefactor Turned 200 on May 19, 1995", Mike Field, the author, contradicts this statement

Chronology, Nursing school

The Institutional Records of The Johns Hopkins Hospital Colored Orphan

Abstract Johns Hopkins Dream for a Model of its Kind: The JHH Colored Orphans Asylum" By Dr. P. Reynolds

"If He Could See Us Now: Mr. Johns Hopkins' Legacy Strong University, Hospital Benefactor Turned 200 on May 19, 1995" by Mike Field a writer for the Johns Hopkins Gazette. Field, Thom, and Jacob called Johns Hopkins an abolitionist. See also The Racial Record of Johns Hopkins University in the Journal of Blacks in Higher Education, No. 25, Autumn, 1999, pp. 42-43/ JSTOR

Johns Hopkins, Maryland State Archives

"The History of African Americans @ Johns Hopkins University" See in particular the chronology and the paper by Danton Rodriguez.

Category:American businesspeople Category:American abolitionists Category:American philanthropists Category:Johns Hopkins University Category:University and college founders Category:People from Baltimore, Maryland Category:People from Anne Arundel County, Maryland Category:American Quakers Category:1795 births Category:1873 deaths