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Eddie L. Patton, M.D., M.S. Yadollah Harati, M.D., F.A.C.P. Unusual Case of Myasthenia Gravis and High White Blood Cell Count. Clinical Presentation. This is the case of a 52 year old man with a 5 week history of left eyelid droop

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Unusual Case of Myasthenia Gravis and High White Blood Cell Count

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Eddie l patton m d m s yadollah harati m d f a c p

Eddie L. Patton, M.D., M.S.

Yadollah Harati, M.D., F.A.C.P.

Unusual Case of Myasthenia Gravis and High White Blood Cell Count


Clinical presentation

Clinical Presentation

  • This is the case of a 52 year old man with a 5 week history of left eyelid droop

  • During this same period of time he noticed some weakness in his arms and legs with exercise.

  • Other intermittent symptoms include double vision, head drop, difficulties swallowing and breathing (all of which are worse in the evening)

  • He has also lost 14 pounds in the previous 4 weeks before presentation to clinic which he attributed to a 1 month history of diarrhea


History

History

  • Past Medical History

    • Diabetes Mellitus 2

    • Diabetic peripheral neuropathy

    • Hyperlipidemia

  • Surgical history

    • Appendectomy

  • Medications- Metformin, ASA, Avandia, Zetia, Cymbalta


History1

History

  • Family history

    • Father: Coronary artery disease

    • Mother: Cancer

  • ROS: Positive for weight loss and diarrhea


Physical exam

Physical exam

  • Cranial nerves

    • Weak palatal elevation

    • Diplopia with lateral gaze

    • Bilateral ptosis

  • Reflexes- Normal

  • Sensation

    • Vibration decreased in the feet up to the ankles, hands normal

    • Pin prick/Light touch- decreased in bilateral toes

    • Temperature- decreased in feet up to ankles


Physical exam1

Physical exam


Physical exam2

Physical exam

  • Most of the muscles tested were easily fatigable

  • Positive ice pack test

  • Clinical diagnosis was pretty clear!


Other tests

Other tests

  • CT Chest- No thymus identified

  • AChR binding antibodies- > 80 H (>0.40)

  • Anti-striational antibodies- 1:320 H (Neg<1:40)


Unusual case of myasthenia gravis and high white blood cell count

  • Diagnosis: Myasthenia gravis

  • Treatment:

    • Six plasma exchanges over 14 day period

    • Improved with initial PE so continued weekly boosters for 4 weeks

    • Started on Imuran

    • Mestinon

  • This is when the case gets more interesting!


Unusual case of myasthenia gravis and high white blood cell count

  • About 1 month after his initial presentation he returned for follow up

    • Diarrhea had returned which he was experiencing on and off over past 4 months

    • Some worsening of ptosis and generalized weakness.

    • Ordered some labs

    • Reduced Mestinon

    • Decided to continue his plasma exchange boosters


Review of labs

Review of labs

  • Initial visit- WBC 37.48 (H)

  • One month later- WBC 14.96 (H)

  • Paraneoplastic panel- Negative

  • Normal liver function tests

  • Mildly elevated glucose


Unusual case of myasthenia gravis and high white blood cell count

  • Three days after this last visit he called office complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • What he didn’t tell us was that a week before his office visit his general practitioner gave him Ciprofloxacin and Metronidazole for the diarrhea and high WBC


Hospital course

Hospital course

  • Pt was admitted to the ICU for MG crisis and subsequently intubated due to respiratory failure

  • Cardiac complications

    • Maintained an increased heart rate

    • Troponins were elevated

    • EF was found to be between 35-39%

    • Diagnosed as having a non-ST elevation myocardial infarction

      • Placed on beta blockers and ACE inhibitors


Hospital course1

Hospital course

  • He received 5 days of IVIG with a good response


Unusual case of myasthenia gravis and high white blood cell count

  • Any ideas on what made this patient worse…


Hosptal course

Hosptal Course

  • Hematological condition

    • During hospital course his hemoglobin began to drop and reticulocyte count was found to be high

    • Peripheral blood smear and flow cytometry was performed

      • 63% monoclonal B cells with immunophenotype positive for CD19, CD23, CD5, CD20, CD38, CD43,CD79b, kappa

    • CLL FISH analysis- Insertional deletion on the proximal arm of one copy of Chromosome 13 consistent with CLL


Final diagnosis

Final Diagnosis

  • Myasthenia gravis crisis

  • CLL

  • Autoimmune hemolytic anemia

  • Non-ST elevation myocardial infarction


Treatment

Treatment

  • He was treated with Vincristine, Cytoxan and stared on Rituximab by hematology

  • He was also started on oral Prednisone to treat his hemolytic anemia

  • After discharge he received 3 weekly Rituximab infusions with good results and was continued on Cytoxan for several months

  • At last appointment his CLL and myasthenia gravis were in remission

    • Only treatment for MG- Mestinon 30-60 mg every 4 hours


Cll and mg

CLL and MG

  • Cohen and Waxman published a case in 1967 Archives of Internal Medicine

  • 28 year old man who was diagnosed clinically with myasthenia gravis in 1930

  • 30 years later in 1960, after a bout of cervical lymphadenopathy, he was diagnosed with CLL

  • In 1964, he presented with hepatosplenomegaly, pulmonary embolization, thrombophlebitis and was found to have a Coomb’s positive hemolytic anemia


Cll and mg1

CLL and MG

  • The authors point to a defect in lymphoid differentiation and the thymus as the linking factor to all these conditions


Cll and autoimmune disorders

CLL and Autoimmune Disorders

  • Conley et al in 1980- “Genetic Factors Predisposing to Chronic Lymphocytic Leukemia and to Autoimmune Disease” sought to prove there was a connection

  • 30 patients with CLL and 28 controls who had some other chronic hematological disorder

  • Pedigrees were constructed from in-depth family histories and medical record reviews

    • Over 320 relatives


Cll and autoimmune disorders1

CLL and Autoimmune Disorders

  • “ A hypothesis given support by our observation is that a genetically conditioned disorder of immune regulation predisoses both to autoimmune disease and to lymphocytic leukemia and other lymphoid tumors”

  • Autoimmune disorders occurred in the families of about 25% CLL patients

Conley,” Genetic Factors Predisposing to Chronic Lymphocytic Leukemia and to Autoimmune Disease” Medicine 1980


Cll and mg2

CLL and MG

  • Bennett et al., “Myasthenia Gravis and Chronic Lymphocytic Leukemia” Eur. J. Hemotology 1989

  • 62 y/o woman diagnosed with B-cell CLL

  • 10 months after being treated with chlorambucil and prednisone developed symptoms consistent with MG

  • Positive edrophonium test and anti-ACh R antibodies high at 33.7 pmol/ml

  • Question raised of defect in immunoregulation


Cll and autoimmune diseases

CLL and Autoimmune Diseases

  • 2005 Fujimaki et al, “Idiopathic thrombocytopenia purpura and myasthenia gravis after fludarabine treatment for chronic lymphocytic leukemia”

  • Up to this point only 10 reported cases of MG in CLL

  • They presented a case of a 60 year old man with WBC count of 30.2 and with 80 % atypical lymphocytes


Fujimaki et al

Fujimaki et al

  • He was diagnosed by cytogenetic studies to have B-CLL

  • Treatment Fludaradine 20 mg/m² IV 5 days every 4-5 weeks

  • After 6 cycles, decrease in WBC (8), no improvement in lymphadenopathy

  • Bone marrow aspiration lead to diagnosis of ITP

  • Two months later he was diagnosed with MG based on diplopia and high anti-Ach receptor antibodies at 79


Fujimaki et al1

Fujimaki et al

  • He was initially treated with Mestinon and 3 days of IV Methylprednisolone, followed by 2 days of plasma exchanges

  • One month later he was started on Rituximab with good response

  • This was the first reported case of CLL associated with ITP and MG

  • Speculation was that the Fludarabine caused further immune dysregulation leading to development of myasthenia gravis and ITP


Summary

Summary

  • Keeping in mind the association between myasthenia gravis and autoimmune disorders and the association between CLL and autoimmune disorders, more extensive work-up may be appropriate in MG patients with hematologic abnormalities

  • Exact mechanism between this association is unknown but immune dysregulation is a very strong theory


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