Crystal-storing histiocytosis

A rare disorder involving histiocytes storing crystalline material

Crystal-storing histiocytosis (CSH) is a rare disorder characterized by the accumulation of histiocytes that contain crystalline material. This condition is often associated with plasma cell dyscrasias and other lymphoproliferative disorders.

Pathophysiology

A macrophage, a type of histiocyte

CSH occurs when histiocytes, a type of immune cell derived from monocytes, accumulate abnormal crystalline structures within their cytoplasm. These crystals are typically composed of immunoglobulin light chains, which are produced in excess in certain disorders such as multiple myeloma and lymphoplasmacytic lymphoma. The accumulation of these crystals can disrupt normal cellular function and lead to tissue damage.

Clinical Presentation

The clinical presentation of CSH can vary widely depending on the organs involved. Commonly affected sites include the bone marrow, liver, spleen, lymph nodes, and kidneys. Patients may present with symptoms related to organ dysfunction, such as renal impairment, hepatosplenomegaly, or lymphadenopathy.

Diagnosis

The diagnosis of CSH is typically made through a combination of clinical evaluation, imaging studies, and histopathological examination. A biopsy of the affected tissue will reveal histiocytes with intracytoplasmic crystalline inclusions.

Diagram of an antibody, showing light chains

Differential Diagnosis

The differential diagnosis for CSH includes other conditions that can cause histiocytic proliferation or crystal deposition, such as Gaucher's disease, Niemann-Pick disease, and amyloidosis.

Treatment

Treatment of CSH focuses on addressing the underlying disorder that is causing the overproduction of immunoglobulin light chains. This may involve chemotherapy, immunotherapy, or other targeted therapies depending on the specific hematological condition.

Prognosis

The prognosis of CSH is largely dependent on the underlying disease. In cases where the associated plasma cell dyscrasia or lymphoproliferative disorder is treatable, the prognosis may be more favorable. However, if the underlying condition is aggressive or resistant to treatment, the prognosis may be poor.

Related pages

• Histiocyte
• Plasma cell dyscrasia
• Multiple myeloma
• Lymphoplasmacytic lymphoma