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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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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 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


Review of labs
Review of labs follow up

  • 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 complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • 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 complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • He received 5 days of IVIG with a good response


Unusual case of myasthenia gravis and high white blood cell count


Hosptal course
Hosptal Course complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • 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 complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • Myasthenia gravis crisis

  • CLL

  • Autoimmune hemolytic anemia

  • Non-ST elevation myocardial infarction


Treatment
Treatment complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • 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 complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • 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 complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • 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 complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • 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 complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • “ 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 complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • 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 complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • 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 complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • 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 complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • 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 complaining of further progression of his swallowing and breathing difficulties, slurred speech and he was admitted to the hospital.

  • 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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