058 ANTI-CASPR2-ANTIBODY associated encephalitis in a 63-year old male with chronic lymphocytic leukaemia

2018 ◽  
Vol 89 (6) ◽  
pp. A24.1-A24
Author(s):  
Kate Lilley ◽  
Andrew Swayne ◽  
Emily Watson ◽  
Rachel Kee ◽  
David Wong ◽  
...  
Keyword(s):  
Chronic Lymphocytic Leukaemia ◽  
Lymphocytic Leukaemia ◽  
Limbic Encephalitis ◽  
Case Reports ◽  
Clinical Status ◽  
Inpatient Rehabilitation ◽  
Ivig Therapy ◽  
Sleep Apnoea ◽  
List Type ◽  
Paraneoplastic Limbic Encephalitis

IntroductionThis case from a tertiary neurology centre reports a novel association between the emerging clinical entity of anti-contactin associated protein-2 (CASPR-2) antibody encephalitis and chronic lymphocytic leukaemia (CLL).CaseWe describe a 63 year old Maori male truck-driver who presented with progressive altered personality, speech, cognition and perception over 9 months. The patient also developed choreiform movements, broad-based gait, incontinence, sleep apnoea with type 2 respiratory failure and episodic loss of consciousness. 12 months prior, he had been diagnosed with low-risk CLL, for which he remained untreated. MRI of the brain revealed mid-sagittal bilateral mid-temporal T2/FLAIR hyperintensities. Cerebrospinal fluid examination showed a mononuclear pleocytosis (WCC 270×10^6/L) with 15% of these CD5/CD19/CD23 positive and 92% CD3/CD5 positive CD 19 negative on flow cytometry, protein was also elevated at 2600 mg/L. The interplay between CLL and inflammation is uncertain. Anti-Caspr2-antibody was identified in CSF and serum. The patient was treated with a combination of fludarabine, cyclophosphamide, rituximab, dexamethasone and intravenous immunoglobulin (IVIG). Clinical status improved and antibody titre decreased from 580 to 241 pM in three weeks and to 55pM (negative <85 pM) at five months. Symptoms worsened when IVIG doses were missed. He returned home after inpatient rehabilitation, showed striking clinical improvement at 12 month follow-up and continues on maintenance IVIG therapy.ConclusionWhilst paraneoplastic VGKC encephalitis has been described associated with a number of malignancies, this is the first reported case of CASPR-2 antibody present in association with CLL.References. Van Sonderen A, Petit-Pedrol M, Dalmau J, Titulaer MJ. The value of LGI1, Caspr2 and voltage-gated potassium channel antibodies in encephalitis. Nature Reviews Neurology2017;13(5):290–301.. Nogai H, Israel-Willner H, Zschenderlein R, Pezzutto A. Improvement in Paraneoplastic Limbic Encephalitis after Systemic Treatment with Rituximab in a Patient with B-Cell Chronic Lymphocytic Leukaemia. Case Reports in Haematology2013;2013:Article ID958704.. Van Sonderen A, Ariño H, Petit-Pedrol M, et al. The Clinical Spectrum of Caspr2 antibody-associated disease. Neurology2016;87:521–528.. Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, et al. Paraneoplastic limbic encephalitis: Neurological symptoms, immunological findings and tumour association in 50 patients. Brain2000;123:1481–1494.

scholarly journals Obstructive sleep apnoea in a patient with chronic lymphocytic leukaemia

2019 ◽  
Vol 12 (7) ◽  
pp. e228763
Author(s):  
Gregory Paul Stimac ◽  
Asefa Jejaw Mekonnen
Keyword(s):  
Chronic Lymphocytic Leukaemia ◽  
Obstructive Sleep Apnoea ◽  
Lymphocytic Leukaemia ◽  
Oral Appliance ◽  
Sleep Apnoea ◽  
Caucasian Woman ◽  
Upper Airways ◽  
Airway Narrowing ◽  
Obstructive Sleep ◽  
Structural Abnormalities

A 69-year-old Caucasian woman presented with chronic lymphocytic leukaemia (CLL; stage 1-Rai System), significant oropharyngeal lymphoid enlargement, snoring and fatigue. Overnight polysomnography revealed moderately severe obstructive sleep apnoea (OSA), which was managed successfully with oral appliance therapy with resolution of snoring and daytime fatigue. Structural abnormalities of the upper airways are known to cause OSA. Airway narrowing can result from bony structural abnormalities, nasopharyngeal growth, soft tissue redundancy, macroglossia, malignant and benign growth of the upper aero-digestive tract, and adenotonsilar enlargement. Clinicians should be encouraged to consider a diagnosis of OSA in patients with CLL when they present with symptoms of worsening fatigue.

scholarly journals Immunoglobulin Replacement Therapy in Chronic Lymphocytic Leukaemia patients with hypogammaglobulinaemia and infection; analysis of total national utilisation data in Australia 2008-2013

2022 ◽  
Author(s):  
Anastazia Keegan ◽  
Peta Dennington ◽  
Nina Dhondy ◽  
Stephen P. MULLIGAN
Keyword(s):  
Chronic Lymphocytic Leukaemia ◽  
Replacement Therapy ◽  
Bacterial Infections ◽  
Lymphocytic Leukaemia ◽  
Supply And Demand ◽  
Ivig Therapy ◽  
Red Cross ◽  
Distribution Data ◽  
Pre Treatment ◽  
Utilisation Data

Objectives To analyse total national utilisation of immunoglobulin (Ig) replacement therapy (IgRT) for Chronic Lymphocytic Leukaemia patients with acquired hypogammaglobulinaemia and severe and/or recurrent bacterial infections. Methods In 2007, the National Blood Authority first published Criteria for the clinical use of intravenous immunoglobulin in Australia. The Australian Red Cross Lifeblood assessed, approved, and recorded all supply with patient demographics, distribution data, intravenous Ig (IVIg) volumes and treatment episodes. IVIg was the sole product used in Australia from 2008-2013 inclusive. Results From 2008-2013 across Australia, 2,734 individual CLL patients received 48,870 treatment episodes using a total 1,324,926 grams of IVIg therapy. Six IVIg products were available, with domestically manufactured Intragam P accounting for 89.7% of supply. The average age for first dose was 74 years. Males received 60.6% of the total treatment episodes representing 20% more than females. The average pre-treatment IgG level was 4.03g/L +/- 2.03g/L (range 0.30-10.50g/L). A sustained average annual increased IVIg utilisation of 5.5% was observed. There was significant regional variation consistent with differences in prescriber preferences across states and territories. Conclusion This study provides a globally unique insight into IgRT supply and demand in CLL patients by analysis of total national use in Australia over a six year period.

Autologous T lymphocytes recognize the tumour-derived immunoglobulin VH-CDR3 region in patients with B-cell chronic lymphocytic leukaemia

2000 ◽  
Vol 111 (1) ◽  
pp. 230-238 ◽  
Author(s):  
Mohammad Reza Rezvany ◽  
Mahmood Jeddi-Tehrani ◽  
Hodjattallah Rabbani ◽  
Ulla Ruden ◽  
Lennart Hammarstrom ◽  
...  
Keyword(s):  
T Lymphocytes ◽  
Chronic Lymphocytic Leukaemia ◽  
B Cell ◽  
Lymphocytic Leukaemia ◽  
Cell Chronic Lymphocytic Leukaemia ◽  
Cdr3 Region

Pharmacokinetics of Chlorambucil in Patients with Chronic Lymphocytic Leukaemia: Comparison of Different Days, Cycles and Doses

2000 ◽  
Vol 87 (5) ◽  
pp. 223-228 ◽  
Author(s):  
Raija Silvennoinen ◽  
Kimmo Malminiemi ◽  
Outi Malminiemi ◽  
Erkki Seppala ◽  
Juhani Vilpo
Keyword(s):  
Chronic Lymphocytic Leukaemia ◽  
Lymphocytic Leukaemia

The Local Sanarelli-Shwartzman Phenomenon in Rats Induced by Human Leukaemic Cells

1973 ◽  
Vol 29 (02) ◽  
pp. 353-362
Author(s):  
J Lisiewicz ◽  
A Pituch ◽  
J. A Litwin
Keyword(s):  
Chronic Lymphocytic Leukaemia ◽  
Intravenous Injection ◽  
Peripheral Blood ◽  
Lymphocytic Leukaemia ◽  
Acute Myeloblastic Leukaemia ◽  
Demarcation Line ◽  
E Coli ◽  
Leukaemic Cells ◽  
Shwartzman Phenomenon

SummaryThe local Sanarelli-Shwartzman phenomenon (SSP-L) in the skin of 30 rats was induced by an intr a cutaneous sensitizing injection of leukaemic leucocytes isolated from the peripheral blood of patients with chronic lymphocytic leukaemia (CLL), acute myeloblastic leukaemia (AL) and chronic granulocytic leukaemia (CGL) and challenged by an intravenous injection of 100(μ of E. coli endotoxin. SSP-L was observed in 7 rats after injection of CLL lymphocytes and in 6 and 2 rats after AL myeloblasts and the CGL granulocytes, respectively. The lesions in the skin after AL myeloblasts appeared in a shorter time and were of longer duration compared with those observed after CLL lymphocytes and CGL granulocytes. Histologically, the lesions consisted of areas of destruction in the superficial layers of the skin ; the demarcation line showed the presence of neutrophils, macrophages and erythrocytes. Haemorrhages and fibrin deposits near the demarcation line were larger after injection of CLL lymphocytes and AL myeloblasts than after CGL granulocytes. The possible role of leucocyte procoagulative substances in the differences observed have been discussed.

Prognostic value of absolute monocyte count in chronic lymphocytic leukaemia

2015 ◽  
Vol 156 (15) ◽  
pp. 592-597
Author(s):  
László Szerafin ◽  
János Jakó ◽  
Ferenc Riskó
Keyword(s):  
Chronic Lymphocytic Leukaemia ◽  
Prognostic Value ◽  
Lymphocytic Leukaemia ◽  
Treatment Time ◽  
Monocyte Count ◽  
Time To Treatment ◽  
Causes Of Mortality ◽  
The Absolute ◽  
The Impact ◽  
Overal Survival

Introduction: The low peripheral absolute lymphocyte and high monocyte count have been reported to correlate with poor clinical outcome in various lymphomas and other cancers. However, a few data known about the prognostic value of absolute monocyte count in chronic lymphocytic leukaemia. Aim: The aim of the authors was to investigate the impact of absolute monocyte count measured at the time of diagnosis in patients with chronic lymphocytic leukaemia on the time to treatment and overal survival. Method: Between January 1, 2005 and December 31, 2012, 223 patients with newly-diagnosed chronic lymphocytic leukaemia were included. The rate of patients needing treatment, time to treatment, overal survival and causes of mortality based on Rai stages, CD38, ZAP-70 positivity and absolute monocyte count were analyzed. Results: Therapy was necessary in 21.1%, 57.4%, 88.9%, 88.9% and 100% of patients in Rai stage 0, I, II, III an IV, respectively; in 61.9% and 60.8% of patients exhibiting CD38 and ZAP-70 positivity, respectively; and in 76.9%, 21.2% and 66.2% of patients if the absolute monocyte count was <0.25 G/l, between 0.25–0.75 G/l and >0.75 G/l, respectively. The median time to treatment and the median overal survival were 19.5, 65, and 35.5 months; and 41.5, 65, and 49.5 months according to the three groups of monocyte counts. The relative risk of beginning the therapy was 1.62 (p<0.01) in patients with absolute monocyte count <0.25 G/l or >0.75 G/l, as compared to those with 0.25–0.75 G/l, and the risk of overal survival was 2.41 (p<0.01) in patients with absolute monocyte count lower than 0.25 G/l as compared to those with higher than 0.25 G/l. The relative risks remained significant in Rai 0 patients, too. The leading causes of mortality were infections (41.7%) and the chronic lymphocytic leukaemia (58.3%) in patients with low monocyte count, while tumours (25.9–35.3%) and other events (48.1 and11.8%) occurred in patients with medium or high monocyte counts. Conclusions: Patients with low and high monocyte counts had a shorter time to treatment compared to patients who belonged to the intermediate monocyte count group. The low absolute monocyte count was associated with increased mortality caused by infectious complications and chronic lymphocytic leukaemia. The absolute monocyte count may give additional prognostic information in Rai stage 0, too. Orv. Hetil., 2015, 156(15), 592–597.

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