"Crystal-storing histiocytosis is rare. There is no consistent sex predilection, with reported series showing a roughly equal sex distribution or a very slight male predominance, although disease associated with autoimmune conditions shows a female predominance 1,2,3,6. It occurs across a wide age range, from 18-91 years, with most patients presenting in the sixth or seventh decade (reported median and mean ages of 60-62 years) 1,2,3."
"Crystal-storing histiocytosis is rare. There is no consistent sex predilection, with reported series showing a roughly equal sex distribution or a very slight male predominance, although disease associated with autoimmune conditions shows a female predominance 1,2,3,6. It occurs across a wide age range, from 18-91 years, with most patients presenting in the sixth or seventh decade (reported median and mean ages of 60-62 years) 1,2,3."
"T1: slightly low signal"
"T2: moderately high signal, with surrounding vasogenic oedema and only minimal mass effect"
"FLAIR: serpentine flow-void-like foci within the lesion, thought to represent prominent veins"
"T1 C+ (Gd): serpentine enhancement, reported in the centrum semiovale"
"MR spectroscopy: elevated choline peak, reduced N-acetylaspartate (NAA) peak, and presence of lactate"
"MR perfusion: mildly increased regional cerebral blood volume and blood flow, with reduced mean transit time and time to peak"
"Crystal-storing histiocytosis was first described by Glaus in 1917 2. Because the distended histiocytes resemble Gaucher cells morphologically and on special stains, they were historically termed pseudo-Gaucher cells, and subsequently pseudo-pseudo-Gaucher cells to distinguish them from the pseudo-Gaucher cells seen in chronic myeloid leukaemia 2."
"Crystal-storing histiocytosis was first described by Glaus in 1917 2. Because the distended histiocytes resemble Gaucher cells morphologically and on special stains, they were historically termed pseudo-Gaucher cells, and subsequently pseudo-pseudo-Gaucher cells to distinguish them from the pseudo-Gaucher cells seen in chronic myeloid leukaemia 2."
"Crystal-storing histiocytosis was first described by Glaus in 1917 2. Because the distended histiocytes resemble Gaucher cells morphologically and on special stains, they were historically termed pseudo-Gaucher cells, and subsequently pseudo-pseudo-Gaucher cells to distinguish them from the pseudo-Gaucher cells seen in chronic myeloid leukaemia 2."
"Crystal-storing histiocytosis is rare. There is no consistent sex predilection, with reported series showing a roughly equal sex distribution or a very slight male predominance, although disease associated with autoimmune conditions shows a female predominance 1,2,3,6. It occurs across a wide age range, from 18-91 years, with most patients presenting in the sixth or seventh decade (reported median and mean ages of 60-62 years) 1,2,3."
"A minority of cases (approximately 10-12%) occur with non-neoplastic conditions, including autoimmune disease (most often Sjögren syndrome and rheumatoid arthritis), infection (notably Helicobacter pylori gastritis), and drugs (notably clofazimine) 2,3."
"Recognition of crystal-storing histiocytosis can be clinically valuable, as it may lead to early identification of an otherwise occult lymphoproliferative or plasma cell disorder 2,3.There is no treatment directed at the histiocytosis itself; management targets the underlying disorder or causative agent 2. For example, treatment of an underlying infection such as Helicobacter pylori gastritis, or withdrawal of a causative drug such as clofazimine, or systemic therapy directed at the associated neoplasm 2."
"The lesion is composed of sheets or aggregates of histiocytes distended by eosinophilic crystalline cytoplasmic inclusions, ranging from needle-shaped to globular 2,3. The inclusions may be PAS-positive or PAS-negative and are characteristically negative on Congo red, which helps distinguish them from amyloid 2."
"The lesion is composed of sheets or aggregates of histiocytes distended by eosinophilic crystalline cytoplasmic inclusions, ranging from needle-shaped to globular 2,3. The inclusions may be PAS-positive or PAS-negative and are characteristically negative on Congo red, which helps distinguish them from amyloid 2."
Expected headings
"Associations"
"Crystal-storing histiocytosis is rare. There is no consistent sex predilection, with reported series showing a roughly equal sex distribution or a very slight male predominance, although disease associated with autoimmune conditions shows a female predominance 1,2,3,6. It occurs across a wide age range, from 18-91 years, with most patients presenting in the sixth or seventh decade (reported median and mean ages of 60-62 years) 1,2,3."
"Presentation is highly variable and depends on the site or sites involved. In approximately a quarter of cases, the condition is found incidentally or as a mass or swelling on examination or imaging 2. Organ-specific manifestations include gastrointestinal symptoms (abdominal pain, diarrhoea, weight loss, bleeding), a non-productive cough with pulmonary involvement, renal impairment, neurological symptoms with brain involvement, and ophthalmic symptoms such as proptosis or reduced visual acuity 2. Because multiple myeloma is the commonest association, many patients also present with myeloma-related features such as bone pain, pathological fractures, hypercalcaemia, anaemia, and renal dysfunction 2."
"Almost any organ can be involved, and localised disease is more common than generalised 2. Overall, the most frequently affected sites are the bone marrow, kidney, lung, lymph nodes, skin, and eye 2. Localised disease tends to favour the head and neck region and the lungs and pleura, whereas generalised disease typically involves the bone marrow, lymph nodes, liver, and spleen 6. Within the gastrointestinal tract, the stomach is most often involved 2. Central nervous system involvement is rare, and within it, the brain is the most frequently affected site 2,4."
"The crystal-laden histiocytes are positive for CD68 and CD163, confirming their histiocytic nature, and are negative for S100, CD1a, langerin, CD138, desmin, smooth muscle actin, and myoglobin 2,3. In neoplastic disease, the crystals are typically monotypic, most often kappa light chain restricted, although lambda restriction also occurs 1,2."
"chest (pleura and lung): mass-forming disease, for example, an extrapulmonary paraspinal soft-tissue mass merging with pleural thickening and without bone destruction; pulmonary lesions may mimic and be resected as carcinoma 2,6"
"Recognition of crystal-storing histiocytosis can be clinically valuable, as it may lead to early identification of an otherwise occult lymphoproliferative or plasma cell disorder 2,3.There is no treatment directed at the histiocytosis itself; management targets the underlying disorder or causative agent 2. For example, treatment of an underlying infection such as Helicobacter pylori gastritis, or withdrawal of a causative drug such as clofazimine, or systemic therapy directed at the associated neoplasm 2."
"History and etymology"