Next Article in Journal
A 80-Year-Old Woman with B-Cell Prolymphocytic Leukemia
Previous Article in Journal
Hemoglobin Willamette (b51 Pro → Arg): Case Report and Literature Review
 
 
Search for Articles:
Title / Keyword
Author / Affiliation / Email
Journal
Article Type
 
 
Advanced Search
 
Section
Special Issue
Volume
Issue
Number
Page
 
Journals
Hematology Reports
Volume 9
Issue 1
10.4081/hr.2017.6859
hematolrep-logo
Submit to this Journal Review for this Journal Propose a Special Issue
► Article Menu

Article Menu

share Share announcement Help format_quote Cite question_answer Discuss in SciProfiles thumb_up
...
Endorse textsms
...
Comment
Hematology Reports is published by MDPI from Volume 14 Issue 1 (2022). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
first_page
settings
Order Article Reprints
Font Type:
Arial Georgia Verdana
Font Size:
Aa Aa Aa
Line Spacing:
Column Width:
Background:
Case Report

Hemophagocytic Lymphohistiocytosis, an Unclear Nosologic Entity: Case Report of an Adult Man with Rising of Amylase and Lipase and Spinal Cord Infiltration

by
Moris Sangineto
*,
Antonio Perrone
,
Pasquale Agosti
,
Viera Boccuti
,
Anna Campobasso
and
Carlo Sabbà
Department of Interdisciplinary Medicine, University of Bari “Aldo Moro”, Piazza Giulio Cesare 11, 70124 Bari, Italy
*
Author to whom correspondence should be addressed.
Hematol. Rep. 2017, 9(1), 4-7; https://doi.org/10.4081/hr.2017.6859
Submission received: 21 September 2016 / Revised: 27 November 2016 / Accepted: 5 December 2016 / Published: 1 March 2017

Abstract

Here we present the case of a 57-years old patient affected by hemophagocytic lymphohistiocytosis (HLH), a rare disease characterized by an uncontrolled immune activation, resulting in clinical and biochemical manifestations of extreme inflammation. In a previous hospitalization, the patient showed fever, hepato-splenomegaly, pancytopenia, hyperferrtitinemia, lymphadenopathy and cholestasis. No diagnosis was done, however, he totally recovered after splenectomy. Eight months later, he relapsed, showing also hypofibrinogenemia, hypertriglyceridemia, hemophagocytic signs in bone marrow, cholestatic jaundice, high LDH and high PT-INR. Interestingly, he presented increased levels of amylase and lipase in absence of radiologic signs of pancreatitis. He was treated with Dexamethasone and Cyclosporine according to HLH-2004 guidelines. The clinical and biochemical manifestations disappeared in a few weeks, but he was newly hospitalized for lower limbs hypotonia caused by a hemophagocytic lesion of the cauda equina and lumbar cord. The death occurred in a few days, despite the immunosuppressive treatment.
Keywords: hemophagocytic lymphohistiocytosis; spinal cord; amylase and lipase; splenectomy hemophagocytic lymphohistiocytosis; spinal cord; amylase and lipase; splenectomy

Share and Cite

MDPI and ACS Style

Sangineto, M.; Perrone, A.; Agosti, P.; Boccuti, V.; Campobasso, A.; Sabbà, C. Hemophagocytic Lymphohistiocytosis, an Unclear Nosologic Entity: Case Report of an Adult Man with Rising of Amylase and Lipase and Spinal Cord Infiltration. Hematol. Rep. 2017, 9, 4-7. https://doi.org/10.4081/hr.2017.6859

AMA Style

Sangineto M, Perrone A, Agosti P, Boccuti V, Campobasso A, Sabbà C. Hemophagocytic Lymphohistiocytosis, an Unclear Nosologic Entity: Case Report of an Adult Man with Rising of Amylase and Lipase and Spinal Cord Infiltration. Hematology Reports. 2017; 9(1):4-7. https://doi.org/10.4081/hr.2017.6859

Chicago/Turabian Style

Sangineto, Moris, Antonio Perrone, Pasquale Agosti, Viera Boccuti, Anna Campobasso, and Carlo Sabbà. 2017. "Hemophagocytic Lymphohistiocytosis, an Unclear Nosologic Entity: Case Report of an Adult Man with Rising of Amylase and Lipase and Spinal Cord Infiltration" Hematology Reports 9, no. 1: 4-7. https://doi.org/10.4081/hr.2017.6859

APA Style

Sangineto, M., Perrone, A., Agosti, P., Boccuti, V., Campobasso, A., & Sabbà, C. (2017). Hemophagocytic Lymphohistiocytosis, an Unclear Nosologic Entity: Case Report of an Adult Man with Rising of Amylase and Lipase and Spinal Cord Infiltration. Hematology Reports, 9(1), 4-7. https://doi.org/10.4081/hr.2017.6859

Article Metrics

Back to TopTop