Showing posts with label lymphopaenia. Show all posts
Showing posts with label lymphopaenia. Show all posts
What is your risk of developing PML on fingolimod?

What is your risk of developing PML on fingolimod?

The following update on PML risk on fingolimod was released to us by Novartis. 


Will this new data lead to a change in practice? 


PML under fingolimod therapy not attributed to previous natalizumab treatment is very rare and the risk is estimated to be less than 1:10,000 patients.

19 confirmed PML cases in >225,000 fingolimod-treated patients (>508,000 patient-years) as of the 30th of November 2017.

The estimated risk (95% CI) is 0.084 (0.051; 0.131)/1,000 patients and the incidence rate (95% CI) is 3.74 (2.25; 5.84)/100,000 patient-years exposure.)
  • Two cases had confounding medical conditions (1 previous cancer and 1 ulcerative colitis/immunosuppressive therapy).
  • One patient had previous NTZ exposure for 10 months (3 years and 9 months before PML diagnosis).
  • In one patient, PML occurred during 3 months of NTZ exposure, after 4.5 years of fingolimod treatment; this patient also had a history of recent exposure to steroids.
  • Demographics: age range from 34 to 71 years, with a female preponderance (14 of 19 cases) and a diverse geographic distribution.
  • Fingolimod exposure ranged from 18 to 84 months. 18 of the 19 patients had fingolimod ranging between 29 and 84 months while one had received fingolimod for 18 months.
  • There was no pattern of sustained grade 4 lymphopenia (defined as absolute lymphocyte count ≤200 cells/µL and based on the reported absolute lymphocyte count values in 15 of 19 cases).
  • JCV DNA PCR test was positive in all cases.

In the past, there has been no clear correlation between opportunistic infection risk and lymphocyte counts on fingolimod. As a result of this, the FDA did not mandate the monitoring of lymphocyte counts in pwMS on fingolimod in routine clinical practice. In comparison, the EMA implemented the same requirements that were part of the fingolimod trial programme; i.e. dose interruption was done if the total, or absolute, lymphocyte count dropped below 200. The latter is quite common in people with MS on fingolimod. To reconcile the differences between the FDA and EMA we suggested a half-way house mark and to only interrupt fingolimod dosing if the counts dropped below 100. 

Only the minority of PML cases (~20%) on fingolimod had counts below 200. Does this mean we now have to change this recommendation? I think it would seem reasonable to go back to the 200 cut-off as recommended by the EMA. 

Does this mean that the FDA will now have to change their recommendations? I am sure they will. The FDA is a data-driven organization and are likely to respond to this new information. 

Unfortunately, fingolimod does not move from the list of DMTs that can't be derisked regarding immunosuppression and opportunistic infections, to the list of DMTs that can potentially be de-risked. The majority of opportunistic infections still occur in pwMS with lymphocyte counts above 200. 

NOTE: If you are on fingolimod and your lymphocyte count is below 200 please speak to your neurologist or clinical nurse specialist. If you don't know your lymphocyte count you should find out. As we can't derisk fingolimod you must be please remain vigilant of any new symptoms, this includes neurological and systemic symptoms, that could suggest an opportunistic infection. 

ProfG    

Predicting low lymphocyte counts after DMTs

How important is it to predict lymphopenia in pwMS starting DMTs? 





Mult Scler Relat Disord. 2017 Dec 14;20:51-57. doi: 10.1016/j.msard.2017.12.003. [Epub ahead of print]

Predictors of hematological abnormalities in multiple sclerosis patients treated with fingolimod and dimethyl fumarate and impact of treatment switch on lymphocyte and leukocyte count.

Baharnoori M, Gonzalez CT, Chua A, Diaz-Cruz C, Healy BC, Stankiewicz J, Weiner HL, Chitnis T.

Abstract

BACKGROUND:

There is limited data regarding the predictors of hematological abnormalities in multiple sclerosis (MS) patients treated with dimethyl fumarate (DMF) or fingolimod (FNG), and the impact of treatment switch on lymphocyte and leukocyte count METHODS: We identified 405 patients on DMF and 300 patients on FNG (treatment duration: at least 12 month) within a large prospective study of MS patients conducted at the Partners MS Center, Brigham and Women's Hospital (CLIMB study) between Jan 2011 to Feb 2016. Patients had complete blood counts with differentials at baseline and every 6 months while on treatment. Most participants had a clinical visit with complete neurologic examinations every 6 months and brain MRI scan every 12 months. T cell subset profile was available for subgroup of patients (n = 116).

RESULTS:

In the FNG group, the risk of developing lymphopenia grade 4 (< 200) was higher in female patients (p = 0.0117) and those who were previously treated with natalizumab (p = 0.0116), while the risk of lymphopenia grade 3b+4 (< 350) was higher in female patients (p = 0.0009). DMF treated patients with lower baseline lymphocyte count had a higher chance of developing lymphopenia grade 2 (< 800) (p < 0.0001) or 2+3 (< 500) (p < 0.0001). We examined the effect of treatment switch between DMF and FNG. No significant recovery in lymphocyte and leukocyte count was observed after treatment switches. Reduced dosing of FNG in patients with lymphopenia led to increase in lymphocyte count but also increased disease activity in 25% of patients.

CONCLUSION:

Female sex and prior exposure to natalizumab increased the probability of lymphopenia on FNG, while low absolute lymphocyte count was associated with increased risk of lymphopenia on DMF. Parallel switch did not lead to recovery from hematological abnormalities. Long-term studies with larger number of patients are required to confirm our findings and to establish guidelines for prediction and management of hematological abnormalities.



Our understanding of treatment-relates side effects in MS, in particular that of immunosuppression, at times seems to deify probability. I suppose if we were privy to the answers, we wouldn't need to predict the antecedent events that lead to it in the first place. The word prediction in itself implies that there is a degree of accuracy involved, but again this would be inaccurate. Baharnoori et al., have attempted to predict the factors involved in treatment related lymphopenia (low lymphocyte count) in their article, so we'll see.

Lymphopenia leaves the person affected by it exposed to opportunistic infections (from colds to pneumonia) and even possibly cancers in the long-term. Drugs, in particular dimethyl fumarate, DMF (aka tecfidera) and fingolimod (aka gilenya) result in severe lymphopenia with lymphocyte counts <500/mm3. Lymphopenia is graded as follows: Grade 1 - 800/mm3; Grade 2 - 800-500/mm3; Grade 3 - 500-200/mm3; and Grade 4 - <200/mm3. Although, in the Phase III clinical trials of DMF Grade 3 lymphopenia was observed in 4% of those who received the treatment, real-life data now shows this figure to be much higher ~20%, particularly in those who are older. The lymphopenia is also persistent in those experiencing Grade 2, or Grade 2+3 lymphopenia with little recovery over the long-term. With fingolimod, there is ~70% reduction in lymphocyte counts (around a quarter develop Grade 4 lymphopenia) within the first month, which then continues for the course of the treatment.


In this study, they found that the main predictors of profound lymphopenia (Grade 4) on fingolimod treatment was female gender (uncertain whether this is a statistical blip as MS is more prevalent in women than men) and prior use of natalizumab. The latter finding has implications as fingolimod is often used as de-escalation therapy from highly active therapies. Reducing the dose of fingolimod to lessen this effect appears to lead to a resurgence in disease activity. A lower absolute lymphocyte count before starting treatment (although this did not reach statistical significance with fingolimod in this study) was also predictive of likelihood of reaching >Grade 2 lymphopenia. It goes without saying, that switching between therapies with known long-term impact on lymphocyte counts is a risky strategy (see Figure below)!


Figure: Absolute lymphocyte count (ALC) (A) and white blood cell (WBC) (B) count on reduced dose of FNG over 42 month (6 month interval). Yearly ALC (C) and WBC (D) on FNG and after switch to DMF. Redline corresponds to the baseline ALC and WBC prior starting FNG. Yearly ALC (E) and WBC (F) on DMF and after switch to FNG. Redline corresponds to the baseline ALC and WBC.