Munchausen syndrome in hematology: Case reports of three variants and review of the literature

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<ul><li><p>Ann Hematol (1994) 68:303-306 9 Springer-Verlag 1994 </p><p>J. Zahner 9 W. Schneider </p><p>Munchausen syndrome in hematology: Case reports of three variants and review of the literature </p><p>Received 14 February 1994 / Accepted 20 April 1994 </p><p>Summary We describe five patients with hematologic Munchausen syndrome who were discovered within 5 years. The patients presented for hematologic evalua- tion of coagulopathy, iron-deficiency anemia, or macro- hematuria with severe anemia. The final diagnoses were phenprocoumon intake (2 x ), self-inflicted phle- botomy (2 ), and urogenital manipulation (1 x ). All five patients were female ranging in age between 23 and 45 years; two were nurses. In four cases we were able to unmask the patient. We think that hematologic Munchausen syndrome has a rather uniform pattern and may be divided into three subtypes: (a) anticoagu- lation type, (b) anemia type, and (c) pretended hemato- logic form. Hematologic Munchausen syndrome should especially be considered if there is an unexplained coa- gulation disorder or therapy-resistant iron-deficiency anemia, and if the patient is a young female nurse who has had many hospitalizations. </p><p>Key words Munchausen syndrome 9 Factitious disor- der 9 Hematology" Coagulation disorder 9 Iron-defi- ciency anemia </p><p>Introduction </p><p>Munchausen syndrome is a chronic factitious disorder with physical symptoms. It was first described by Asher in 1951 and named after the German Baron Mtinch- hausen [4]. This historical figure is well known for his travels and his formidable lies, which inspired Asher to call patients with self-induced illness who have many hospitalizations Munchausen patients. These patients often tell untruthful stories and confabulations and fre- quently wander from hospital to hospital. In many </p><p>J. Zahner (l~) 9 W. Schneider Klinik far H~imatologie, Onkologie und klinische Immunologie, Heinrich-Heine-Universit~tt, Moorenstrasse 5, D-40225 Dt~sseldorf, Germany </p><p>cases, female nurses are involved. Patients mostly live as singles and seem to have been emotionally deprived since childhood [38]. Many invasive procedures and tests are performed in these patients without pathologic result. As soon as the patient is suspected of being a Munchausen patient, he leaves the hospital and looks for another clinic. </p><p>Meadow [22] described a special entity of Mun- chausen syndrome in childhood, called Munchausen syndrome by proxy: Parents, mostly the mother, fabri- cate symptoms in their children. It is important to men- tion that Munchausen syndrome by proxy is a psychiat- ric disease of the parents and that the young patient is abused. This form of child abuse is associated with a mortality of 9% for the children and often involves he- matologic features [24, 37]. </p><p>The main variants of Munchausen syndrome differ- entiated by Asher are "laparotomophilia migrans", which means the patient goes around looking for oper- ations, "neurologia phantastica", which refers to neuro- logic symptoms and "haemorrhagica histrionica", which means that the patient very impressively tells stories about blood loss, which is self-induced. Nearly one third of adult Munchausen patients have hematologic symptoms [8, 27]; in Munchausen syndrome by proxy even two thirds demonstrate hematologic features [23]. </p><p>Case reports </p><p>Patient 1. A 25-year-old shop assistant was admitted to the hospi- tal because of epistaxis. We found normal values for thrombo- cytes (350000/ixl), but low Quick values (&lt; 10%) and prolonged PTT ( &gt; 2 min). Analysis of coagulation factors showed a decrease of vitamin-K-dependent factors. Although we were able to de- monstrate high levels of phenprocoumon in her serum, the pa- tient strongly denied taking any anticoagulants. On contacting her local doctor we learned about five hospitalizations within 2 years. During these stays the suspicion had arisen that the patient had manipulated her surgical wounds. Further inquiries gave evidence of a disturbed personality and severe family conflicts. After thor- ough questioning the patient admitted anticoagulant intake. </p></li><li><p>304 </p><p>Patient 2. A 28-year-old nurse complained about fatigue and ex- haustion. The only pathologic findings was microhematuria, which had been detected in several urine analyses. Multiple en- doscopies (cystoscopy, colonoscopy, gastroscopy) had been per- formed without pathologic results. She had been treated for iron deficiency for several years with oral and parenteral iron substitu- tion but without long-lasting success. Therefore, erythrocytes had to be transfused. Although she had been treated in several hospi- tals, the cause of iron deficiency had not been detected. As the patient was working in a laundry and had come into contact with aromatic substances, toxic bone marrow damage was suspected. We unmasked the patient after we found phlebotomy needles in her bedside table. Psychologic exploration showed a damaged personality structure. </p><p>Table 1 Three subtypes of hematologic Munchausen syndrome </p><p>1. Real bleeding disorder caused by - coumarin - aspirin </p><p>2. Real anemia caused by - phlebotomy - urogenital manipulation - rectal manipulation </p><p>3. Bleeding disorder or anemia pretended by - localized manipulations - patient's statement - Munchausen syndrome by proxy </p><p>Patient 3. A 45-year-old nurse had suffered from hemorrhagic cys- titis for many years. Although macrohematuria had been diag- nosed many times in the past, cystoscopy had provided no patho- logic findings. The patient was admitted to our hospital because of a severe microcytic, hypochromic anemia with Hb of 5.7 g/dl. Serum ferritin was very low, and bone marrow biopsy showed iron-deficiency anemia. No source of blood loss was detected with endoscopic procedures. The patient refused oral iron substitution and transfusion of erythrocytes. When we confronted her with our suspicion of manipulating her urinary tract, she left the hospi- tal. During a further hospitalization in a different clinic, a urinary catheter was found in her bedside table and the patient admitted to self-manipulation of her bladder. </p><p>Discussion </p><p>These three patients show the spectrum of hematologic Munchausen syndrome. Patient 4, a 31-year-old wo- man, took phenprocoumon, patient 5 was 23 years old and performed phlebotomy. Patient 5 is the only one who was available for follow-up. Within 28 months aft- er dismission she had ten further admissions to four hospitals. During these stays she displayed the same symptoms as in our hospital. To our knowledge, this patient had no suicidal thoughts, which are frequently reported in the long run of Munchausen syndrome [8]. Despite our efforts, the other four patients were lost to follow-up. </p><p>The histories of patients 4 and 5 are not noted here, because they are similar to cases 1 and 2. Self-induced coagulation disorder and iron-deficiency anemia are the most frequent symptoms in hematologic Munchaus- en syndrome. Patients are not restricted to hematologic symptoms, however; they simultaneously may pretend nephrologic (case 2), urologic (case 3), and cardiac symptoms and produce factitious surgical illness by wound manipulation (case 1). Although the patients' personalities seemed disturbed, we did not see psy- chiatric factitious disease in these five cases. The lack of psychiatric factitious disease in hematologic Munchaus- en patients is in accordance with the literature [8]. On reviewing the literature and looking at our own data, we think that hematologic Munchausen patients may be divided into three groups (Table 1). </p><p>Anticoagulation type </p><p>Hematologic factitious disorder was first reported by Stafne and Moe in 1951, the same year in which Asher created the name "Munchausen syndrome" for chronic factitious disorders [32]. They described a 26-year-old nurse who had petechiae and ecchymoses due to self- administered dicumarol intake, which, however, was denied. Agle et al. reported on three nurses between 27 and 30 years of age who had deliberately ingested anti- coagulants [2]. Psychiatric study of these patients showed multiple neurotic symptoms and hysterical and masochistic character traits. Several other authors have described anticoagulant intake as a variant of factitious disease [5, 7, 13, 15, 20, 25, 26]; Bowie [7] coined the expression "dicumarol eaters". Bleeding disorders caused by anticoagulants may not only lead to epistaxis as in case 1; ecchymosis, menorrhagia, hemoptysis, he- maturia, gingival bleeding, or melena may also occur. Factitious bleeding may be combined with other non- hematologic Munchausen features [17, 20]. The anti- coagulant is taken either as a tablet or in the form of rat poison [20]. Even if the patient is unmasked, new epi- sodes of ingestion may follow [5]. Coumarin intake in the Munchausen syndrome must be carefully differen- tiated from accidental overdose during therapy, inad- vertent intake by a pharmacist or patient, and ingestion in order to commit suicide or murder [31]. The most frequent cause probably is deliberate self-administra- tion in Munchausen syndrome. Diagnosis is usually made by determination of the anticoagulant in the pa- tient's plasma. </p><p>Aside from coumarin intake, there have been only rare cases of other anticoagulants in the Munchausen syndrome. A patient with colonic angiodysplasia took high doses of aspirin which led to severe anemia. Al- though a high serum aspirin concentration was mea- sured, the patient denied anticoagulant intake but stop- ped self-medication for the future [18]. </p><p>Anemia type </p><p>After coagulation disorders, anemia is the main symp- tom in hematologic Munchausen. The first descriptions of this variant were reported by Daily et al. [11] and </p></li><li><p>305 </p><p>Bernard et al. [6]; the latter described 12 women be- tween 20 and 43 years of age with self-induced anemia. As shown in our case 2, anemia is mostly provoked by phlebotomy [1, 3, 16, 34-36]. In rare cases local uroge- nital (case 3), oral [33], or rectal manipulation [6] is ob- served. Iron depletion can nearly always be demon- strated in the bone marrow, and serum ferritin is low. Despite oral iron substitution Hb does not increase, and so intravenous iron substitution or transfusion of erythrocytes is applied. Compared with self-induced coagulation disorders, the diagnosis of anemia is more difficult and cannot be achieved in all suspected cases. Diagnosis usually is made by finding phlebotomy nee- dles or catheters in the patient's bedside table. But even then diagnosis is still questionable if the patient does not admit manipulation with these tools. </p><p>Pretended hematologic Munchausen </p><p>Some patients do not suffer from bleeding disorder or anemia, but claim to suffer from such diseases as hemo- philia [10], acute myelogenous leukemia, and systemic lupus erythematosus [29]. Other Munchausen patients pretend local bleeding in the form of hematuria [9, 28], hematemesis [3, 9], hemoptysis [8, 14], or melena [3, 9] by manipulating their urine, saliva, or stools with their own blood or blood from other patients. In some cases patients simulate hematemesis or melena by venesect- ing themselves and ingesting their own blood [3, 9]. Factitious purpura and bruising of the skin have been reported in a few cases [12, 21]. Recurrent severe epis- taxis has also been described [30]. In these patients he- matologic Munchausen syndrome meets with other forms of Munchhausen disease such as dermatologic, ENT, nephrologic, urologic, or gastrointestinal mani- festations (case 3). </p><p>Alleged bleeding or anemia is quite often found in Munchausen syndrome by proxy. Bloody stools, blood in the diaper, bloody emesis, and bleeding from the res- piratory tract are faked by the mother with her own blood [24]. In some cases blood typing and 51Cr-label- ing of the child's erythrocytes proved that the blood did not derive from the child, but was maternal [19, 37]. </p><p>As there is a uniform pattern concerning the cardi- nal symptoms in hematologic Munchausen syndrome, hematologists should be aware of the most common types of this entity (Table 1). Unfortunately, definite diagnosis of hematologic Munchausen, especially of the anemia type, is difficult and cannot be achieved in all suspected cases. Although we made every effort, only three patients conceded self-induced illness in hour hospital. The fourth was unmasked and admitted to her manipulations during a stay in another hospital. The fifth did not admit to phlebotomy, although a needle was found in her bedside table. As generally observed in factitious disorders, there is a strong predominance of female nurses also in hematologic Munchausen. All </p><p>of our five patients were younger women, and two of them had worked as nurses. </p><p>If Munchausen syndrome in a patient is suspected, but not proven, physicians should be reluctant to per- form invasive diagnostic procedures which may harm the patient. Especially biopsies from multiple tissues such as liver, kidney, or bone marrow bear the risk of complications. </p><p>Once a diagnosis of Munchausen syndrome is made, patients tend to withdraw from the physician and the hospital very quickly. Only a minority of patients are willing to undergo psychotherapy. Results from therapy of Munchausen syndromes are poor. In only a few cases has psychotherapy prevented the patient from continu- ing her self-inflicted illness [35]. In most cases all medi- cal and psychologic efforts are in vain. </p><p>We think hematologic Munchausen syndrome should be taken into consideration when bleeding or coagulation disorders are present which cannot be ex- plained otherwise. Particularly if the patient is a young female nurse with psychosocial abnormalities and a his- tory of many hospitalizations, a hematologic Mun- chausen syndrome should be suspected. </p><p>Acknowledgements We thank Dr. Andreas H. Heer-Sonderhoff for his critical review of the manuscript. </p><p>References </p><p>1. Abram H, Hollender M (1974) Factitious blood disease. South Med J 67:691~696 </p><p>2. Agle D, Ratnoff O, Spring G (1970) The anticoagulant malin- gerer - psychiatric studies of three patients. Ann Intern Med 73: 67-72 </p><p>3. Anderson J, Ewan P, Compston N (1972) Haemorrhage and fever in the Munchausen syndrome. Post Med J 48:445-447 </p><p>4. Asher R (1951) Munchausen's sydnrome. Lancet 1:339-341 5. Bartels H, Franke B, Lutze G (1990) Wiederholte Selbstbe- </p><p>sch~idigung durch die Einnahme eines Cumarinderivates. Z Arztl Fortbild 84: 665-666 </p><p>6. Bernard J, Najean Y, Alby N, Rain J (1967) Les anemies hy- pochromes dues a des hemorragies volontairement provo- quees. Syndrome de Lasthenie de Ferjol. Presse Med 75 : 2087-2090 </p><p>7. Bowie E, Todd M, Thompson J00wen C, Wright I (/965) Anticoagulant malingerers (the "dicumarol eaters"). Am J Med 39 : 855-864 </p><p>8. Carney M, Brown J (1983) Clinical features and motives among 42 artificial illness patients. Br J Med Psychol 56:57- 66 </p><p>9. Chugh K (1966) Haemorrhagica histrionica - the bleeding Munchausen syndrome. J Indian Med Assoc 46:90-93 </p><p>10. Cottam S, Cuthbert C, Parapia L (1991) Munchausen AIDS and hae...</p></li></ul>