RECORD OF PROCEEDINGS 
PHYSICAL DISABILITY BOARD OF REVIEW

NAME: XXXXX 		BRANCH OF SERVICE: AIR FORCE 
CASE NUMBER:  PD0900385		BOARD DATE: 20100721
SEPARATION DATE: 20050301 
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SUMMARY OF CASE:  This covered individual (CI) was a Staff Sergeant, Computer Systems Operator medically separated from the Air Force in 2005 after 6 years and 7 months of service.  The medical basis for the separation was Thrombocytosis associated with gastroesophageal reflux from Agrylin therapy.  The CI was referred to the Physical Evaluation Board (PEB), determined unfit for the Thrombocytosis condition, and separated at 10% disability using the Veterans Affairs Schedule for Ratings Disabilities (VASRD) and applicable Air Force and Department of Defense regulations.
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CI CONTENTION:  The CI states: “The VA has rated me at 100% for the main service-connected condition that I was discharged for (Essential Thrombocytosis).  After I was discharged the VA did genetic testing on me, and it was determined that I suffered a JAK2 genetic mutation during my military service.  I have been told by every hematologist or oncologist doctor seen that this condition is a life-long condition that I must be treated for. Treatment that was started during my military service included the use of a very strong anti-platelet drug (generic name of anagrelide) that puts me at risk of developing acute leukemia, amongst other things.  The official medical description that the VA has evaluated me under is Thrombocytosis with Leukocytosis and Neutrophilia.  This condition has many possible effects on my body, such as heart attack, stroke, and the chance that it may morph into acute leukemia or myelofibrosis.  There is no cure for my condition.”
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RATING COMPARISON: 

Service PEB  	VA (4 Months before Separation)	
Condition	Code	Rating	Date	Condition	Code	Rating	Exam	Effective	
Thrombocytosis Associated With Gastroesophageal Reflux From Agrylin Therapy.
	7704-7799	10%	20040928	Thrombocytosis With Leukocytosis And Neutrophilia	7799-7704	100%

100%	20041116

20070307	20050302	
		NARSUM
(Fracture)	Rhinitis with  Healed Nasal Fracture	6502-6522	10%	20041116	20050302	
		Not in DES	Acne	7828	10%	20041116	20050302	
		Not in DES	Headaches	8100	10%	20041116	20050302	
		Not in DES	Strain of the Thoracolumbar Spine	5237	0%	20041116	20050302	
		NARSUM
(Surgery)	Residual Scar Right Lower Quadrant from Appendectomy	7805	0%	20041116	20050302	
TOTAL Combined:  10%	TOTAL Combined (Includes Non-PEB Conditions):    
100%  from 20050302

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ANALYSIS SUMMARY:
A Medical Evaluation Board (MEB) Narrative Summary (NARSUM) completed 20040521 reported the CI was first noted to have elevated platelets in Feb 2001, at 689,000/µL.  She was referred to hematology and a bone marrow biopsy was performed in April 2001, which showed mildly hypercellular marrow with no evidence of malignancy and normal stainable storage iron.  Peripheral smear at that time showed Leukocytosis with Neutrophilia and Thrombocytosis.  She was followed by Dr L. in Italy, who noted no need to treat in Oct 2001.  She was referred in Jan 2003 to hematology for further workup and definitive diagnosis.  Over the next few months she was seen by Dr H. and started first on Plavix and baby aspirin, but platelets remained relatively high. Although there were no signs or symptoms suggestive of bleeding or thrombosis, she was started on Agrylin 0.5mg by mouth twice a day.  This was a more aggressive treatment to lower the platelet count.  She was also given Gaviscon and Pepcid AC both for the resulting Gastroesophageal Reflux Disease (GERD) symptoms since starting the Agrylin.  Her most recent (prior to the NARSUM) platelet count on 7 April 04 was 405,000/µL (while on Agrylin.) Due to the continuing Thrombocytosis, it was recommended that she stay well hydrated and stay out of potential problems with dehydration by avoiding high temperatures.  Also while on the Agrylin she required close monitoring of complete blood counts and side effects of the medication.  Follow-up visits every six months were recommended.  An MEB was initiated in accordance with AFI 48-123 A2.8.8. She was separated with a 10% rating under 7099-7704.  No rationale for this rating was in the available records.

Follow-up and lab tests continued after the MEB NARSUM had been completed in May 2004.  Lab tests from 20040903 and 20041006 both showed normal white cell and red cell counts, hematocrit, and hemoglobin.  Platelet count was slightly elevated at 421,000/µL in September and 459,000/µL in October (normal was listed as 154-402,000/µL).  She underwent a complete cardiac work-up for frequent palpitations, dizziness, and chest discomfort.  She had a normal EKG, a normal stress thallium, and a normal echocardiogram (except for mild tricuspid regurgitation).  The thyroid stimulating hormone level was also normal in October 2004.  Her symptoms were determined to be related to her use of Agrylin and not due to a cardiac problem. Her palpitations improved with Atenolol. 

There are no reports of any signs or symptoms of either bleeding or thrombosis in the service treatment record (STR) and the CI’s platelet count was below 459,000/µL on multiple occasions.  The Agrylin was controlling her platelet count and preventing bleeding and thrombosis. 

An initial VA Compensation and Pension (C&P) examination was done 20041116, four months prior to separation from service.  It reported all the symptoms she had experienced but it was not clearly documented if they were still present or had resolved. Her platelets were noted to be 479,000/µL, mild/ high.  The actual lab report was not available so it was not clear what her white cell and red cell counts were at that time.  But they were normal in October 2004 as reported above.  They were also normal at a later VA test on 20070305.

There is no VASRD code for Thrombocytosis and the VA rated the CI’s condition analogous to 7704 Polycythemia Vera.  This is reasonable as Polycythemia Vera causes increased red blood cells and sometimes also causes increased white blood cells and platelets.  Polycythemia Vera is a malignancy and Thrombocytosis is not.  In addition to other medications, Agrylin is sometimes also used to decrease elevated platelet counts in Polycythemia Vera.  With elevated platelets there is risk of both bleeding and thrombosis.  Agrylin prevents platelet aggregation and inhibits megakaryocyte maturation, thereby decreasing platelet counts. Scientific studies have shown it to be efficacious in reducing platelet counts in both Polycythemia Vera and Thrombocytosis and to be safe with respect to leukemic transformation.  The most frequent side effects are headache, palpitations, and diarrhea.  Agrylin does not lower the number of red or white blood cells and therefore does not increase the risk of anemia or infection.

The VA rating of 100% was based on a diagnosis of Thrombocytosis with Leukocytosis and Neutrophilia; reported lightheadedness, headaches, fatigability, weakness, shortness of breath at rest, sensitivity to light, heart palpitations, racing heart, chest pain, and back pain; and treatment with myelosuppressants.  This was considered a temporary rating and a future exam was scheduled for November 2006.  While Leukocytosis with Neutrophilia was noted at the time of the bone marrow biopsy in 2001, subsequent lab tests documented a normal number of leukocytes.

The follow-up VA C&P exam was done 20070307. This exam also did not separate a review of systems from the history of present illness and reported multiple symptoms.  She was still taking the Agrylin and she now reported intermittent claudication after walking one mile on level ground at two miles per hour.  She had no calf pain at rest but reported persistent coldness of her extremities.  Her extremities had no edema on examination.  She also reported easy bleeding but had no bruising or petechiae on her skin.  Her physical exam was normal and her labs of 20070305 showed a platelet count of 516,000/µL, hemoglobin of 14.0g/dL, and hematocrit of 40.8%.  VA treatment records showed that her Agrylin was being tapered and the plan was to discontinue it if her platelet levels remained reasonable.

Anagrelide (Agrylin) is used to decrease the number of platelets (a type of blood cell that is needed to control bleeding) in the blood of patients who have a myeloproliferative disorder (condition in which the body makes too many of one or more types of blood cells) such as Essential Thrombocythemia (also called Essential Thrombocytosis; condition in which the body makes too many platelets) or Polycythemia Vera (condition in which the body makes too many red blood cells and sometimes too many platelets).  Anagrelide is in a class of medications called platelet-reducing agents.  It works by slowing the production of platelets in the body. It does not lower the number of red blood cells or white blood cells as stated in the VARD 20050309.

Agrylin does not decrease the number of red blood cells or cause anemia.  However, some of the side effects of the medication are similar to symptoms one might have with anemia.  These include headache, lightheadedness or dizziness, lack of energy or sleepiness, weakness, flu-like symptoms, and fainting.  It can also cause muscle, joint or back pain, leg cramps, chest pain, fluttering sensation in the chest, fast, forceful, or irregular heartbeats, swelling of the arms, hands, feet, ankles or lower legs, weakness or numbness of an arm or leg, pain, burning, or tingling in the hands or feet, and changes in vision.

Essential Thrombocytosis (primary Thrombocythemia) is characterized by a platelet count greater than 600,000/µL, megakaryocytic hyperplasia, splenomegaly, and a clinical course complicated by thrombotic and/or hemorrhagic episodes.  The CI never had evidence of any episodes of hemorrhage or thrombosis or hepatosplenomegaly.  These conditions were successfully prevented through use of the platelet lowering medication, Agrylin. Her platelet count had been as high as 863,000/µL in October 2003, but remained near 400,000 and below through April 2004 while on Agrylin.  In September and October 2004 her platelet count was slightly higher at 421,000/µL and 459,000/µL respectively. 

At the time of separation The CI’s condition was stable on medication and while one of her complaints (headache) could be due to Thrombocytosis, all of her symptoms reported on the VA C&P examination could also (and most could only be) be side effects of her medication, not secondary to the Thrombocytosis. More likely than not, the headache was a side effect of the CI’s medication, as her platelet count was below the diagnostic threshold for thrombocytosis.  Also, the VA C&P exam listed multiple symptoms in the history of present illness but did not specifically address if the symptoms were happening currently or had been present in the past (or both).  There was no separate review of systems and it appears the review of systems was combined with the history of present illness.  The NARSUM examination did include a separate review of systems section and it documented no current symptoms.  Heart palpitations were reported on the NARSUM as past symptoms which had since resolved.  The NARSUM was done in May 2004, six months prior to the VA C&P exam. 

Other Conditions  
Rhinitis with Healed Nasal Fracture and Residual Scar Right Lower Quadrant from Appendectomy

There is no evidence either of these conditions were unfitting at the time of separation from service.  No duty restrictions are attributable to either condition and neither interfered with satisfactory performance of any required duties.

Other Conditions not in the Disability Evalution System (DES)
Acne, Headaches, and Strain of the Thoracolumbar Spine
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BOARD FINDINGS:  IAW DoDI 6040.44, provisions of DoD or Military Department regulations or guidelines relied upon by the PEB will not be considered by the Board to the extent they were inconsistent with the VASRD in effect at the time of the adjudication.  After careful consideration of all available information the Board unanimously determined that the CI’s condition is most appropriately rated at 10% disability as 7799-7704 Thrombocytosis.

As there is no VASRD code specific for Essential Thrombocytosis, it is rated analogous to 7704 Polycythemia Vera.  However, Polycythemia Vera is a malignancy and Thrombocytosis is not.  The CI’s condition was stable on continuous medication, Agrylin, and this warrants a 10% rating.  Her platelet count remained well below the level required for diagnosis, 600,000/µL, and there was no evidence of bleeding or thrombosis.  Agrylin decreases only platelets with no effect on red or white blood cells.  Therefore it does not cause an increased risk of anemia or infection and does not cause the same level of disability as myelosuppressive agents used to treat malignancies.  

The Board also considered the conditions of Rhinitis with Healed Nasal Fracture and Residual Scar Right Lower Quadrant from Appendectomy and unanimously determined that neither was unfitting at the time of separation from service.  No duty restrictions are attributable to either condition and neither interfered with satisfactory performance of any required duties. 

The other diagnoses rated by the VA (Acne, Headaches, and Strain of the Thoracolumbar Spine) were not mentioned in the DES package and are therefore outside the scope of the Board.  The CI retains the right to request her service Board of Correction for Military Records (BCMR) to consider adding these conditions as unfitting.
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RECOMMENDATION: The Board therefore recommends that there be no recharacterization of the CI’s disability and separation determination.

UNFITTING CONDITION	VASRD CODE	RATING	
Essential Thrombocytosis	7799-7704	10%	
COMBINED	10%	
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The following documentary evidence was considered:

Exhibit A.  DD Form 294, dated 20090526, w/atchs.
Exhibit B.  Service Treatment Record.
Exhibit C.  Department of Veterans' Affairs Treatment Record.

                             

SAF/MRB
1535 Command Drive, Suite E-302
Andrews AFB, MD  20762-7002

	Reference your application submitted under the provisions of DoDI 6040.44 (Section 1554, 10 USC), PDBR Case Number PD-2009-00385.

	After careful consideration of your application and treatment records, the Physical Disability Board of Review determined that the rating assigned at the time of final disposition of your disability evaluation system processing was appropriate.  Accordingly, the Board recommended no re-characterization or modification of your separation with severance pay.

	I have carefully reviewed the evidence of record and the recommendation of the Board.  I concur with that finding and their conclusion that re-characterization of your separation is not warranted.  Accordingly, I accept their recommendation that your application be denied.