Crystalglobulinemia with Fulminant Course with Cylinder-like Bodies on Peripheral Blood Smear

Kawaguchi, Takeshi; Kariya, Yumi; Matsuda, Monique; Kubo, Kazuyoshi; Miyauchi, Syunichi; Kusumoto, Norio; Ueno, Shiro; Takajo, Ichiro; Nagatomo, Yasuhiro; Tahira, Yuki; Yamamoto, Shino; Yorita, Kenji; Kataoka, Hiroaki; Okayama, Akira

doi:10.2169/internalmedicine.53.1775

Cited by 7 publications

(17 citation statements)

References 12 publications

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“…The crystals in the present case were immunohistochemically positive for IgG and kappa. The shapes of the crystals in the capillaries of patients with crystalglobulinemia have been reported to be rectangular, square, rhomboid, and needle‐shaped and range in diameter from 5 to 70 µm . Ultrastructurally, the crystal deposits in the present case were needle‐shaped in the renal biopsy and rectangular to rhomboid in the aortic wall examined at autopsy.…”

Section: Discussionmentioning

confidence: 45%

“…The shapes of the crystals in the capillaries of patients with crystalglobulinemia have been reported to be rectangular, square, rhomboid, and needle-shaped and range in diameter from 5 to 70 mm. 1,2,8,9 Ultrastructurally, the crystal deposits in the present case were needle-shaped in the renal biopsy and rectangular to rhomboid in the aortic wall examined at autopsy. Both deposits had fine periodicity of 10 nm, and the fine structure was similar.…”

Section: Discussionmentioning

confidence: 50%

“…Crystalglobulinemia is an extremely rare complication of monoclonal gammopathy . The term “crystalglobulinemia” is applied to cases without evidence of cryoglobulin .…”

mentioning

confidence: 99%

“…1,2 The term "crystalglobulinemia" is applied to cases without evidence of cryoglobulin. 3 However, serum tests for cryoglobulin are occasionally negative in patients with crystalglobulinemia, [2][3][4] and cryoglobulin is not a sole cause of crystalglobulinemia. Nevertheless, the pathological consequences are similar in both diseases: multiple crystal thrombi develop in the capillaries and arterioles of systemic organs.…”

mentioning

confidence: 99%

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Laminar crystal deposition in large vessels in a patient with crystalglobulinemia

Shintani-Domoto

Shinozaki‐Ushiku

Okuma

et al. 2017

Pathology International

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Crystalglobulinemia is an extremely rare complication of monoclonal gammopathy and is characterized by crystal thrombi within systemic organs. We herein report the first described case of crystalglobulinemia accompanied by laminar crystal deposition in the large vessels. A 44-year-old man presented with a history of numbness, pain, and swelling of the left leg in addition to visual impairment. Renal and skin biopsies revealed crystal thrombi within the capillary lumens. The patient was finally diagnosed with crystalglobulinemia associated with multiple myeloma. He was treated with hemodialysis and chemotherapy but died of the disease 15 months after admission. Autopsy demonstrated a huge amount of crystal deposition in the subintimal layer of the vascular wall throughout the thoracic to abdominal aorta. The characteristic deposition extended to the iliac arteries, common carotid arteries, and subclavian arteries but did not affect the bilateral renal arteries. Antemortem computed tomography demonstrated higher intensity in the wall of the abdominal aorta but not in the walls of the renal arteries, suggesting that a finding of high intensity on computed tomography could be a clinical marker of systemic crystal deposition.

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Section: Discussionmentioning

confidence: 45%

Section: Discussionmentioning

confidence: 50%

“…Crystalglobulinemia is an extremely rare complication of monoclonal gammopathy . The term “crystalglobulinemia” is applied to cases without evidence of cryoglobulin .…”

mentioning

confidence: 99%

mentioning

confidence: 99%

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Laminar crystal deposition in large vessels in a patient with crystalglobulinemia

Shintani-Domoto

Shinozaki‐Ushiku

Okuma

et al. 2017

Pathology International

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“…[2] M-protein-related disease caused by pathological immunoglobulins and light chains, such as cryoglobulinemia, POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome, AL amyloidosis, and light-chain deposition disease, can have diverse symptoms such as pyrexia, arthralgia, skin rash, peripheral neuropathy, thrombosis, and renal involvement, which mimic systemic rheumatic diseases. [3] Although M-protein-related diseases can cause fulminant end-organ damage, [4,5] indications for aggressive treatment of MGUS causing M-protein-related disease have not been known. We report a case of crystalglobulinemia, which is a rare form of M-protein-related disease, characterized by irreversible cryoprecipitation, and it is caused by monoclonal gammopathy that was previously diagnosed as MGUS.…”

Section: Introductionmentioning

confidence: 99%

Crystalglobulinemia manifesting as chronic arthralgia and acute limb ischemia

Abe

Tomita²,

Bohgaki³

et al. 2017

Medicine

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Rationale:Crystalglobulinemia is a rare disease caused by monoclonal immunoglobulins, characterized by irreversible crystallization on refrigeration. It causes systemic symptoms including purpura, arthralgia, and vessel occlusive conditions to be exacerbated by exposure to cold. We report a patient with crystalglobulinemia associated with monoclonal gammopathy of undetermined significance (MGUS) manifesting as chronic arthralgia and recurrent acute arterial occlusion.Presenting concerns:A 61-year-old man, who had been diagnosed with MGUS and who had arthralgia of unknown origin, presented with recurrent acute limb ischemia after surgical thromboembolectomy. Refrigeration of his serum formed precipitates that looked like needle-shaped crystals. These crystals did not dissolve with warming, which is not a characteristic of cryoglobulins. Skin biopsy results showed crystal-liked eosinophilic bodies in small vessels and we diagnosed crystalglobulinemia.Intervention and outcomes:Although he underwent above-knee amputation, he was treated with a bortezomib and dexamethasone-based chemotherapeutic regimen, following lenalidomide maintenance therapy. Finally, he achieved complete remission and serum crystalglobulins diminished.Lessons:Monoclonal gammopathy, previously diagnosed as MGUS, can cause systemic symptoms and thrombotic conditions by producing pathologic immunoglobulins, such as crystalglobulins. In such situations, MGUS, even when it has not progressed to multiple myeloma, can be a target of aggressive chemotherapy. Crystalglobulinemia should be considered for patients with monoclonal gammopathy manifesting as systemic and thrombotic symptoms exacerbated by cooling.

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When Monoclonal Gammopathy is of Renal Significance: A Case Study of Crystalglobulinemia From Chicago Multiple Myeloma Rounds

Gilmore

Rodby

Cimbaluk

et al. 2019

Clinical Lymphoma Myeloma and Leukemia

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Crystalglobulinemia with Fulminant Course with Cylinder-like Bodies on Peripheral Blood Smear

Cited by 7 publications

References 12 publications

Laminar crystal deposition in large vessels in a patient with crystalglobulinemia

Laminar crystal deposition in large vessels in a patient with crystalglobulinemia

Crystalglobulinemia manifesting as chronic arthralgia and acute limb ischemia

When Monoclonal Gammopathy is of Renal Significance: A Case Study of Crystalglobulinemia From Chicago Multiple Myeloma Rounds

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