The Shah’s Spleen: Its Impact on History
The spleen had never before played a major role in shapinghuman history. Failing hearts, damaged brains, or organspiercedbyassassins’bulletshadalloccupiedcenterstageintheir time. In 1979, a diseased spleen provoked a seriousconflict between The United States and the newly formedIslamicRepublicofIran,reverberationsofwhicharestillinplay today. In the dramatic evolution of that conflict many other countries played lesser but significant roles. Medicalprofessionalism was challenged in a multinational arena,involving dozens of doctors. In the central role of a haplesspatient in need of urgent surgical treatment was the re-cently deposed Shah of Iran, Mohammad Reza ShahPahlavi.Mohammad Reza Pahlavi became the Shah of Iran inSeptember 1941 at the age of 22, after the forced abdica-tion of his father, Reza Pahlavi, founder of the Pahlavidynasty. Unlike his father, who had a strong, despotic de-meanor, the new Shah assumed the role of a benevolentdespot, whose goals were to modernize the country andguide it into the 20th century. Within 10 years into hisreign, the oil industry was nationalized, education was up-graded by building schools, libraries, and institutions of higher learning, and women’s suffrage was instituted. Thecountry’s infrastructure was strengthened and the plight of the Iranian people was unquestionably improved.
Politically the Shah was decidedly pro-Western in inter-national relations. Physically he was handsome, urbane,andoutwardlyahealthyandactiveindividual,twiceescap-ing assassination attempts and twice surviving crashes inaircrafthewaspiloting.Earlyinhisreignitseemedthathismonarchy would fare well under him. But the Middle Eastis noted for its constantly seething discontent, and Islamicextremists continuously challenged the stability of hisreign. In October 1971, undeterred by the gatheringstorm, the Shah put on a lavish celebration at the ruins of ancient Persepolis, marking the 2500
anniversary of thePersianEmpire.Ifanything,thepompousscaleandextrav-agant cost of the event did nothing but exacerbate thegrowing threat to his rule.In early 1974, The Shah’s spleen entered the picture. WhilevacationingontheisleofKishinthePersianGulfhenoticed an unusual swelling in his left upper abdomen. Hehad been water-skiing (a favorite activity) and the swelling was probably rendered more prominent in the posture as-sociated with that sport. Back in Tehran, his physiciansdetermined that the swelling was an enlarged spleen andsummoned urgent consultation with the French hematol-ogist Dr Jean Bernard, well known for his expertise in he-matologic disorders, particularly lymphoma. Dr Bernardchose to take with him his young pupil, Dr GeorgesFlandrin. So began the keeping of a secret that remained well guarded for the next several years. Even the invitation wasdeceptive.Theywereinvitedostensiblynottoexaminethe Shah, but one of his aides. On their arrival it was madeclear that the patient was the Shah himself.The French doctors examined the Shah and confirmedthepresenceofsplenomegaly.Theyalsodetectedsomecer-vical lymph node enlargement. Blood studies, includingbone marrow, established the diagnosis of chronic lym-phatic leukemia. Although the doctors recommended acervical node biopsy for a tissue diagnosis as well, the Shahbalked at the idea of a surgical procedure. Anxious to sparethe Shah from the anxiety likely to be induced by the word
,theShah’spersonalphysicianconvincedthevisitingFrench doctors to tell the Shah that he had Waldenstrom’s“disease.” The Shah accepted this diagnosis and only learned of his true diagnosis several years later. The Shah,his doctors, and his personal aide all agreed that there wasto be no disclosure of any information even suggestingmalignancytoanyone.TheShah’sconditionwastobekepta well-guarded secret from everyone, even his wife, theQueen Farah. She was to learn of the diagnosis, to herdismay, several years later.The French consultants initially recommended observa-tiononly.Severalmonthslatertheyprescribedaregimenof chlorambucil to keep the disease in check. Although headhered to the chemotherapeutic regimen initially, theShah proved to be an inconsistent and noncompliant pa-tient. On one occasion he even took the wrong medicationasaresultofaprescriptionrefillerror,alsoaconsequenceof the conspiracy to keep the diagnosis secret. In 1975, thedoctorswerecalledagaintomeettheShahinZurich,Swit-zerland.Theretheyfoundthatthespleenwasenlargingandrecommended that the dosage of chlorambucil be in-creased. They also advised that the Shah desist from phys-icalactivitiessuchasskiing,asporthewasinveteratelyfond
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Received September 2, 2010; Accepted October 27, 2010.From the Department of Surgery, David Geffen School of Medicine at Uni-versity of California, Los Angeles, Los Angeles, CA.Correspondenceaddress:LeonMorgenstern,MD,FACS,Cedars-SinaiMed-ical Center, 8700 Becker Blvd, Becker Building - Suite 216, Los Angeles, CA 90048.
© 2011 by the American College of Surgeons ISSN 1072-7515/11/$36.00Published by Elsevier Inc. doi:10.1016/j.jamcollsurg.2010.10.014