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Crystalglobulinemia with fulminant course with cylinder-like bodies on peripheral blood smear
Takeshi Kawaguchi1, Yumi Kariya, Motohiro Matsuda
1Department of Rheumatology, Infectious Diseases, and Laboratory Medicine, Faculty of Medicine, University of Miyazaki, Japan.
Insights
A rare condition called crystalglobulinemia caused a patient's rapid decline. Identifying unique cylinder-like bodies in blood smears can aid in diagnosing this rare hypergammaglobulinemia.
Area of Science:
- Hematology
- Pathology
- Immunology
Background:
- Monoclonal gammopathy is characterized by the overproduction of immunoglobulin.
- Hypergammaglobulinemia can rarely lead to the formation of protein crystals within the bloodstream.
Observation:
- A 63-year-old woman presented with fever, purpura, and leg pain, rapidly progressing to death.
- Peripheral blood smear and skin biopsy revealed distinctive cylinder-like bodies (20×120 μm).
- Autopsy confirmed these crystalline structures within the vasculature of multiple organs.
Findings:
- The patient was diagnosed with monoclonal gammopathy (IgG, lambda type) and bone marrow examination showed atypical plasma cell proliferation, indicative of underlying myeloma.
- Crystalglobulinemia was identified as the cause of widespread small vessel embolisms.
- The presence of cylinder-like bodies in peripheral blood was a key diagnostic finding.
Implications:
- Crystalglobulinemia is a rare but critical diagnosis associated with significant morbidity and mortality.
- Early identification of cylinder-like bodies in blood smears can facilitate timely diagnosis and management of this condition.
- This case highlights the importance of recognizing rare protein crystallization disorders in unexplained embolic events.
Abstract:
A 63-year-old woman presented to our hospital with fever, purpura and pain in both legs and died 4 days after admission. Her blood smear and skin biopsy showed cylinder-like bodies (20×120 μm). She was diagnosed to have monoclonal gammopathy (IgG, lambda type). An autopsy revealed cylinder-like bodies in the vasculature of various organs. We noted a proliferation of atypical plasma cells in her bone marrow, suggesting pre-existing myeloma. Crystalglobulinemia is a rare manifestation of hypergammaglobulinemia that can cause multiple embolisms of the small vessels, and this resulted in the patient's fulminant course. The identification of cylinder-like bodies in the peripheral blood may help in reaching a diagnosis in such cases.
