Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2022 Sep 26;58(10):1350.
doi: 10.3390/medicina58101350.

Secondary Hemophagocytic Syndrome in a Patient with Plasma Cell Myeloma and CNS Involvement Treated with Lenalidomide

Sławomir Milczarek  1   2 , Piotr Kulig  1 , Bartłomiej Baumert  1   2 , Aleksandra Łanocha  2 , Krzysztof Sommerfeld  2 , Ewa Borowiecka  2 , Bogumiła Osękowska  1   2 , Edyta Paczkowska  1   2 , Barbara Zdziarska  2 , Bogusław Machaliński  1   2
Affiliations
Case Reports

Secondary Hemophagocytic Syndrome in a Patient with Plasma Cell Myeloma and CNS Involvement Treated with Lenalidomide

Sławomir Milczarek et al. Medicina (Kaunas). .

Abstract

We present an extremely rare case report of a 29-year-old multiple myeloma patient with central nervous system involvement and secondary hemophagocytic lymphohistiocytosis (HLH). We observed that HLH was presumably triggered by the immunomodulatory drug-lenalidomide. HLH is frequently misdiagnosed or underdiagnosed. As HLH requires immediate treatment, our report emphasizes the need to consider HLH in the differential diagnosis when the condition of a patient receiving chemotherapy rapidly deteriorates and an infectious etiology is excluded. We furthermore discuss the pathogenesis of HLH, with particular emphasis on drugs affecting the immune system as well as possible therapeutic strategies.

Keywords: bone marrow transplantation; chemotherapy; hemophagocytic lymphohistiocytosis; multiple myeloma.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
CT (a,b) and MRI (c,d) scans of plasma cell tumor infiltrating central nervous system (CNS).
Figure 2
Figure 2
Trend chart of HLH-relatedlaboratory tests: (a)ferritin [μ/L]; (b) fibrinogen [mg/dL]; (c) interleukin 6 [pg/mL]; (d) bilirubin [mg/dL]; (e) aspartate aminotransferase [IU/L]; (f) alanine aminotransferase [IU/L]; (g) triglycerides [mg/dL]; (h) c-reactive protein [mg/L]. Each box on the x-axis corresponds to 1 day.
Figure 3
Figure 3
Hemophagocytic lymphohistiocytosis (a–f) (HLH) in patient with plasma cell myeloma. Bone marrow aspirate smear depicting hemophagocytosis, macrophages engulfing erythrocytes and erythroblasts (different magnitudes).
Figure 3
Figure 3
Hemophagocytic lymphohistiocytosis (a–f) (HLH) in patient with plasma cell myeloma. Bone marrow aspirate smear depicting hemophagocytosis, macrophages engulfing erythrocytes and erythroblasts (different magnitudes).
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Siegel R.L., Miller K.D., Jemal A. Cancer statistics, 2016. CA Cancer J. Clin. 2016;66:7–30. doi: 10.3322/caac.21332. - DOI - PubMed
    1. Katzmann J.A., Dispenzieri A., Kyle R.A., Snyder M.R., Plevak M.F., Larson D.R., Abraham R.S., Lust J.A., Melton L.J., 3rd, Rajkumar S.V. Elimination of the Need for Urine Studies in the Screening Algorithm for Monoclonal Gammopathies by Using Serum Immunofixation and Free Light Chain Assays. Mayo Clin. Proc. 2006;81:1575–1578. doi: 10.4065/81.12.1575. - DOI - PubMed
    1. Fassas A.B.T., Muwalla F., Berryman T., Benramdane R., Joseph L., Anaissie E., Sethi R., Desikan R., Siegel D., Badros A., et al. Myeloma of the central nervous system: Association with high-risk chromosomal abnormalities, plasmablastic morphology and extramedullary manifestations: Myeloma of the Central Nervous System. Br. J. Haematol. 2002;117:103–108. doi: 10.1046/j.1365-2141.2002.03401.x. - DOI - PubMed
    1. Filipovich A., McClain K., Grom A. Histiocytic Disorders: Recent Insights into Pathophysiology and Practical Guidelines. Biol. Blood Marrow Transplant. 2010;16:S82–S89. doi: 10.1016/j.bbmt.2009.11.014. - DOI - PubMed
    1. Henter J.I., Horne A., Aricó M., R Egeler M., Filipovich A.H., Imashuku S., Ladisch S., McClain K., Webb D., Winiarski J., et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr. Blood Cancer. 2007;48:124–131. doi: 10.1002/pbc.21039. - DOI - PubMed

Publication types

MeSH terms