Woman fights hemophagocytic lymphohistiocytosis, a rare disease
December 10, 2006
The wife of Grand Forks state Sen. Nick Hacker is fighting a rare blood disease.
Elizabeth Hacker is suffering from hemophagocytic lymphohistiocytosis, or HLH. She became ill less than a month after the two were married in August. Doctors found her blood had trouble clotting.
Elizabeth Hacker, a Langdon native who teaches speech pathology the Larimore elementary school, had blood transfusions and surgery to fight infection, and had been unconscious for weeks. Last week, her family reported some signs of improvement, and she could communicate with her husband for the first time in months.
[...]The virus is diagnosed as HLH only in about 1 out of every million infants. That Hacker is an adult with HLH is even more rare, said Dr. Michael Burke, a specialist in pediatric hematology and oncology at the University of Minnesota Fairview Children’s Hospital.
- Source: AP, via The Bismarck Tribune, Nov. 14, 2006
The Centers for Disease Control and Prevention (CDC) says:
Hemophagocytic lymphohistiocytosis (HLH) is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis (phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors) in bone marrow and other tissues.
- Source: Hemophagocytic Syndromes and Infection, Centers for Disease Control and Prevention
Little Research
The Histiocytosis Association of America writes:
Histiocytosis is a rare blood disease that is caused by an excess of white blood cells called histiocytes. The histiocytes cluster together and can attack the skin, bones, lung, liver, spleen, gums, ears, eyes, and/or the central nervous system. The disease can range from limited involvement that spontaneously regresses to progressive multiorgan involvement that can be chronic and debilitating. In some cases, the disease can be life-threatening.
In some ways, histiocytosis is similar to cancer and has historically been treated by oncologists with chemotherapy and radiation. Unlike cancer, histiocytosis sometimes goes into remission without treatment.
The vast majority of people diagnosed with histiocytosis are children under the age of 10, but it is also found in adults of all ages.
It is approximated that histiocytosis affects 1 in 200,000 children born each year in the United States. This illness is so rare, there is little research into its cause and treatment, and it is often referred to as an “orphan disease,” meaning it strikes too few people to generate government - supported research.
- Source: Disease Information, Histiocytosis Association of America
Two forms of hemophagocytic lymphohistiocytosis
Doctors recognize a primary (familial) and a secondary form of HLH.
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder primarily affecting young children at a very early age, but it is found in patients of all ages. Although physicians have written about the disorder over the years, it has only been recently that it has received much attention.
[...]There are two major forms of HLH. One, known as the primary form, is inherited. The other is known as the secondary form. In the secondary form, the disease develops secondary to inappropriate (abnormal) activity of the immune system. This can occur after the use of immunosuppressive therapy and/or infections.
[...]Familial hemophagocytic lymphohistiocytosis (also known as FHL, FEL or FHLH) means that the particular form of the disease is genetic. Families who have had more than one child with HLH will have the diagnosis changed to FHL. Because this form is genetic, there is a 25% chance that each young sibling will have FHL. The onset of FHL usually occurs within the first two years of life.
- Source: The Facts About HLH and FHL, Histiocytosis Association of America
Support Groups
The Histiocytosis Association of America maintains a networking directory specifically for HLH and FHL families who wish to share information and mutual support.
