Treatment for Hemophagocytic Syndromes

Some patients may be cured with corticosteroid therapy, but immunosuppression is needed to control other complications. In those who develop hematopoietic toxicity from aHDS treatment, autologous stem cell transplantation may be effective. Most patients with aHDS requiring treatment do not respond adequately to the first-line therapy, and subsequent treatment options may be immunosuppression, splenectomy, or autologous or allogeneic stem cell transplantation alone or in combination.

About 15,000 people per year are diagnosed with one or more forms of HLH. This accounts for less than 1% of all hospitalized adult patients in the United States. There are three distinct subtypes of familial hemophagocytic syndromes. Only about 1,000 to 3,000 people per year are diagnosed with HLH of the familial chimaeric form. No cases of HLH attributed to EBV-HLH are seen annually in the United States. The annual number of people with HLH associated with HIV remains uncertain. HLH is uncommon in patients with HIV and, when noticed, rarely causes serious symptoms.

Hemophagocytic syndromes occur mainly in adults but mainly affects children, sometimes resulting in death of the child in the absence of anticoagulants. Hemophagocytic syndromes can occur as a secondary manifestation of autoimmune diseases, such as rheumatic diseases, but can also be the main pathology of some diseases, such as juvenile idiopathic arthritis, and leukemia. It is important to recognize and manage hemophagocytic syndromes in order to prevent severe and even lethal complications.

In this patient population, the major neurologic manifestations were nonspecific. Seizures were found in approximately 10% of patients. The only radiologically evident abnormality was the usual increased leukocyte number on routine chest x-rays, although this finding is not specific to hemophagocytic syndromes.

Hemophagocytic syndromes can not be cured. However, with the advancement of immunosuppressive, antiviral, and antibiotic drug therapies, patients with hemophagocytic syndromes can live happily and maintain their quality of life.

Hemophagocytic syndromes occur after one of the following events: a viral infection (either prior to or during the acute episodes), or an immune-mediated disease (either prior to or during the acute episodes), or a malignancy. This condition is frequently associated with lymphoproliferative disturbances (including immunoblastic lymphoma).

The incidence of Haemophagocytic syndrome is lower than previously thought but more often involves children. It has been found that the average age at the time of diagnosis is higher. This may highlight the importance of early diagnosis and diagnosis of more people with this condition. There is ongoing research to improve diagnosis and treatment of the condition, to decrease the complications. There is potential for more treatments that involve hemophagocytic syndromes as well as the use of the prothrombin complex concentrate, (a-Factor XIII concentrate), intravenous immunoglobulin, and other immunomodulatory therapies when necessary.

The current literature suggests that immunosuppression, chemotherapy, and radiotherapy is associated with a worsening of symptoms for patients with hemo-related disorders. It appears that therapies typically used as single therapies are less likely to be used in combination, though this may be the case for other patients and disorders.

There have been several clinical trials involving treatment for patients with this disease and several patients with hemophagocytic syndrome (a disease similar to hemophagocytic lymphohistiocytosis/HLH) have died within a short time after being treated with various treatments, but it has not been determined if treatment is the cause of the deaths. Patients with hemophagocytic syndromes seem to benefit most from treatment with the agents used in these clinical trials. [Ampheresis, etoposide, and other chemotherapy agents have been used in a few clinical trials for hemophagocytic syndromes when conventional or biologic immunosuppression was inadequate.] [Power](http://www.

It was evident that both treatment and control subjects experienced benefits. Further, these benefits of control and treatment were not significantly different. Results from a recent clinical trial is the first to show that a patient-controlled, medication-overutilization, regimen-based regimen is as effective as a placebo in the treatment of the disorder. We propose that further studies of treatment of this disease need to be a patient-controlled, medication-overutilization, regimen-based regimen.

This first randomized study to assess the efficacy of a leukoreduction filter system in a randomized, controlled trial of patients with aPLDs indicates that rituximab and eculizumab both improve outcome measures. However, while these new drugs treat patients more effectively than the traditional therapies used in the past, patients still experience side effects. More research is needed to assess which medications are most effective for treating thrombotic complications.