scholarly journals Hemophagocytic lymphohistiocytosis, an unclear nosologic entity: case report of an adult man with rising of amylase and lipase and spinal cord infiltration

2017 ◽  
Vol 9 (1) ◽  
Author(s):  
Moris Sangineto ◽  
Antonio Perrone ◽  
Pasquale Agosti ◽  
Viera Boccuti ◽  
Anna Campobasso ◽  
...  

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.

2021 ◽  
pp. 194187442110446
Author(s):  
Matthew R. Woodward ◽  
Margaret S. Ferris ◽  
Guillermo Rivell ◽  
Laura Malone ◽  
Tara M. Dutta ◽  
...  

We are writing to present an interesting and novel case from our practice of a patient who presented with altered mental status and a rapidly progressive paraplegia as well as high fevers and pancytopenia. A bone marrow biopsy was diagnostic of hemophagocytic lymphohistiocytosis (HLH) and MRI showed hemorrhagic encephalitis and spinal subarachnoid hemorrhage. This case demonstrates the diverse neurological symptoms with which HLH presents, including spinal cord pathology. The astute neurologist should consider this diagnosis in the appropriate clinical context and diagnosis may require imaging to the complete neuraxis.


2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Samin Alavi ◽  
Maryam Ebadi ◽  
Alireza Jenabzadeh ◽  
M. T. Arzanian ◽  
Sh. Shamsian

Herein, the first case of childhood erythrophagocytosis following chemotherapy for erythroleukemia in a child with monosomy 7 is reported. A 5-year-old boy presented with anemia, thrombocytopenia, and hepatosplenomegaly in whom erythroleukemia was diagnosed. Prolonged pancytopenia accompanied by persistent fever and huge splenomegaly and hepatomegaly became evident after 2 courses of chemotherapy. On bone marrow aspiration, macrophages phagocytosing erythroid precursors were observed and the diagnosis of HLH was established; additionally, monosomy 7 was detected on bone marrow cytogenetic examination. In conclusion, monosomy 7 can lead to erythrophagocytosis associated with erythroid leukemia and should be considered among the chromosomal abnormalities contributing to the association.


2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S93-S93
Author(s):  
A Rjoop ◽  
M Barukba ◽  
O Al Rusan

Abstract Introduction/Objective Hemophagocytic Syndromes are a cluster of disorders related to cytotoxic dysfunction of T/NK-cells and are mainly subdivided into Primary (familial) and Secondary (acquired) forms, with the latter usually linked to patients with viral infections; including EBV, CMV among many others. A myriad of other causes have been associated with hemophagocytic lymphohistiocytosis (HLH), most notably systemic inflammatory conditions; especially Juvenile Rheumatoid Arthritis and hematolymphoid malignancies particularly T/NK-cell lymphomas. Methods/Case Report A previously healthy 7-year-old boy, presented to the ER with fever and a skin rash over both lower limbs of 1 week duration. Two weeks prior he was tested for COVID-19 and was found to be positive. Physical examination further revealed slightly palpable liver and spleen. CBC was done and exhibited pancytopenia, further testing showed elevated LDH, hyperferritinemia and hypertriglyceridemia. However, serological testing for rheumatological conditions was unremarkable. Imaging studies were done and were noncontributory. Subsequently, a bone marrow aspirate and biopsy were done. The bone marrow aspirate showed afew histiocytes engulfing red blood cells and nuclear debris (hemophagocytic cells), complete trilineage maturation and normal M:E ratio of 3:1. Trephine biopsy was hypocellular for age and estimated at about 70%, composed of myeloid and erythroid precursors with various degrees of maturation. Megakaryocytes were adequate in number and showed normal morphology. Extensive histiocytic infiltration as highlighted by CD68 immunostain and focal phagocytosis were identified. CD34 highlighted <5% blasts, PAS special stain showed no fungal elements and no fibrosis was evident by Reticulin special stain. The background was devoid of lymphoid aggregates or granulomas. Stainable iron stores were depleted. No sideroblasts were identified. The patient was treated with corticosteroid and showed marked improvement and was discharged after 3 days. Results (if a Case Study enter NA) NA Conclusion Hemophagocytic lymphohistiocytosis can be a critical sequela of COVID-19 infection. Suggested mechanisms include impaired/delayed T-cell response and elevated levels of several inflammatory cytokines. Clinical suspesion is important in the diagnosis of these cases. Further study of this correlation is needed as we explore clinical sequelae of COVID-19 infection.


Author(s):  
Ashraf S. Gorgey ◽  
Pamela D. Moore ◽  
Rodney C. Wade ◽  
Ranjodh S. Gill ◽  
Timothy Lavis ◽  
...  

2020 ◽  
Vol 39 (03) ◽  
pp. 228-231 ◽  
Author(s):  
Arthur Lopes ◽  
Kleber Duarte ◽  
Catarina Lins ◽  
Gabriel Kubota ◽  
Valquíria Silva ◽  
...  

AbstractColorectal cancer is one of the most common oncological diseases. Chemotherapy is usually recommended as an adjuvant treatment for stage-II, -III, and -IV tumors. Approximately 10% of the patients develop neuropathic pain after chemotherapy, and they may remain refractory despite the administration of drugs that are commonly used to treat neuropathic pain. Spinal cord stimulation is a good treatment option for neuropathic pain of the lower limbs, and it should be trialed in patients with chemotherapy-induced peripheral neuropathy. We report the case of a patient with oxaliplatin-induced neuropathy and neuropathic pain refractory to oral medication who was successfully treated by spinal cord stimulation.


2014 ◽  
Vol 11 (2) ◽  
pp. 150-153
Author(s):  
Pradipta Tripathy ◽  
Chidananda Dash ◽  
Aurobind Rath ◽  
S. Chakraborty ◽  
Jagannath Sahoo ◽  
...  

2019 ◽  
Vol 20 ◽  
pp. 1299-1304 ◽  
Author(s):  
Phe Phedy ◽  
Yoshi P. Djaja ◽  
Luthfi Gatam ◽  
Yuyus Kusnadi ◽  
Rosiana P. Wirawan ◽  
...  

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Yogesh Subedi ◽  
Utsav Joshi ◽  
Sanjeeb Sudarshan Bhandari ◽  
Ashbina Pokharel ◽  
Ashbita Pokharel

Background. Neuromyelitis optica is a severely disabling inflammatory disorder of the central nervous system of autoimmune etiology that mainly affects the optic nerves and spinal cord. Here, we present a case report detailing a patient with tingling and weakness of right upper and lower limbs who was neuromyelitis optica immunoglobulin G-positive. Case Presentation. A 46-year-old Nepalese man presented to the hospital with a history of tingling and weakness of right upper and lower limbs that developed over a period of two months. Clinical evaluation showed diminished power across all major muscle groups in the right upper and lower limbs. Magnetic resonance imaging of his cervical spine showed T1 iso- to hypointense signal and T2 hyperintense signal in central cervical spinal cord from first to sixth cervical level, probably suggestive of myelitis or demyelination. The patient was immediately started on intravenous methylprednisolone. The diagnosis of neuromyelitis optica was later confirmed with strongly positive neuromyelitis optica immunoglobulin G. Conclusion. In resource limited setting, in the absence of tests for neuromyelitis optica immunoglobulin G, treatment was started and the patient’s condition started to get better. Hence, early initiation of aggressive immunosuppressive treatment is essential in such cases.


2020 ◽  
Vol 121 (1) ◽  
pp. 49-54
Author(s):  
Alireza Tabibkhooei ◽  
Farid Kazemi ◽  
Foad Kazemi ◽  
Morteza Taheri

Tethered cord syndrome (TCS) may rarely remain asymptomatic until degenerative or nondegenerative lumbar diseases superimpose in adulthood and expose the hidden anomaly. In such cases, different treatment options can be selected and simultaneous detethering might be considered too. We are reporting an undiscovered TCS in a young lady who underwent lumbar diskectomy due to symptomatic disk extrusion and suffered complete cauda equina syndrome (CES), postoperatively.


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