Hemophagocytic lymphohistiocytosis- Signs, Symptoms, Diagnosis and Treatment

Journal of Cancer Research and Immuno-Oncology is an open access rapid peer reviewed journal in the field of cancer research. Here we discuss about Hemophagocytic lymphohistiocytosis- Signs, Symptoms, Diagnosis and Treatment.

Hemophagocytic lymphohistiocytosis (HLH), also known as Hemophagocytic lymphohistiocytosis is an uncommon hematologic disorder seen more often in children than in adults. It is a life-threatening disease of severe hyper inflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages, characterized by proliferation of morphologically benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines. It is classified as one of the cytokine storm syndromes. There are inherited and non-inherited (acquired) causes of Hemophagocytic lymphohistiocytosis (HLH).

Signs and symptoms

HLH clinically manifests with fever, enlargement of the liver and spleen, enlarged lymph nodes, yellow discoloration of the skin and eyes, and a rash. Laboratory findings may include elevated triglyceride levels, low fibrinogen levels, transaminitis, and elevated ferritin levels (among others).

Diagnosis

Light microscopic image of bone marrow showing stromal macrophages containing numerous red blood cells in their cytoplasm

The blood count typically shows decreased numbers of blood cells—including a decreased number of circulating red blood cells, white blood cells, and platelets.

The bone marrow may show hemophagocytosis.

The liver function tests are usually elevated. A low level of the protein albumin in the blood is common.

The serum C reactive protein, erythrocyte sedimentation rate, and ferritin level are markedly elevated. In children, a ferritin above 10000 is very sensitive and specific for the diagnosis of HLH, however, the diagnostic utility for ferritin is less for adult HLH patients.  The serum fibrinogen level is usually low and the D-dimer level is elevated.

The sphingomyelinase is elevated.

Bone marrow biopsy shows histiocytosis.

 Treatment

In secondary cases, treatment of the cause, where possible, is indicated. Additionally, treatment for HLH itself is usually required.

While optimal treatment of HLH is still being debated, current treatment regimes usually involve high dose corticosteroids, etoposide and cyclosporin.  Intravenous immunoglobulin is also used. Methotrexate and vincristine have also been used. Other medications include cytokine targeted therapy.

On 20 November 2018, the FDA approved the anti-IFN-gamma monoclonal antibody emapalumab (proprietary name Gamifant) for the treatment of pediatric and adult primary HLH.

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