Lymphohistiocytosis (Hemophagocytic Lymphohistiocytosis) Follow-up

Updated: Mar 20, 2018
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Follow-up

Further Outpatient Care

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  • Supportive care is needed to ensure that the patient with hemophagocytic lymphohistiocytosis (HLH) remains stable until a bone marrow donor can be found. This includes transfusions of RBCs, platelets, and fresh frozen plasma, as well as nutritional support in addition to the treatment protocol. [40]

  • Patients should be continually monitored for evidence of infection because of their immunosuppressed state. Be aware of the possibility of underlying malignancy.

  • Biopsies and diagnostic cytogenetics may be needed in addition to selective radiographic imaging. [22]

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Complications

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  • Complications of individual drugs in the regimen were outlined above (see Medication).

  • Complications due to a subsequent transplant are numerous and include both acute and chronic graft versus host disease, acute inflammatory events, respiratory distress syndrome, and exacerbation of neurologic symptoms. [45]

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Prognosis

Although the prognosis varies between studies and with different approaches to treatment, the disease is invariably fatal if not treated. The median survival rate has been reported to be 2-6 months without treatment, but survival time has dramatically improved with the advent of the HLH-94 protocol already discussed.

A study of 122 patients from the International Registry for hemophagocytic lymphohistiocytosis found that the overall estimated 5-year survival rate was 21%, with 66% of patients who received bone marrow transplantation (BMT) surviving 5 years versus only 10.1% of patients treated with chemotherapy alone. [24] More recent studies have shown that the HLH-94 protocol resulted in an overall survival rate of 55%. Success or failure of an allogeneic BMT is the most important long-term prognostic factor. Unfortunately, many cases are diagnosed late in the course of the disease, after irreversible damage has occurred.

Although patients with hemophagocytic lymphohistiocytosis are at high risk for death early in their disease course, steroids, intravenous immunoglobulin (IVIG), or both may be sufficient as first-line therapy for selected patients. [46]

The ratio of C16 –ceramide to sphingosine was elevated in those who died despite appropriate treatment, but remained low in survivors, implying that this ratio may be of prognostic significance. [47] The balance of ceramide and sphingosine may be pivotal in the clinical outcome for these patients.

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Patient Education

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  • All physicians who treat young patients must be aware of life-threatening diseases such as hemophagocytic lymphohistiocytosis.

  • Pediatricians, dermatologists, and neurologists should especially take note because the presenting symptoms of hemophagocytic lymphohistiocytosis are likely to bring the patient into their offices.

  • Any suspicion warrants a referral to a pediatric hematologic-oncologist who is equipped with the necessary tools to make a rapid diagnosis.

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