Development of secondary myelodysplastic ... - Wiley Online Library

American Journal of Hematology 81:557–561 (2006)

LETTERS AND CORRESPONDENCE

Letters and correspondence submitted for possible publication must be identified as such. Text length must not exceed 500 words and five bibliographic references. A single concise figure or table may be included if it is essential to support the communication. Letters not typed double-spaced will not be considered for publication. Letters not meeting these specifications will not be returned to authors. Letters to the Editor are utilized to communicate a single novel observation or finding. Correspondence is to be used to supplement or constructively comment on the contents of a publication in the journal and cannot exceed the restrictions for Letters to the Editor. The Editor reserves the right to shorten text, delete objectional comments, and make other changes to comply with the style of the journal. Permission for publication must be appended as a postscript. Submissions must be sent to Jay Umbreit, MD, PhD, Editor of Brief Reports/Letters to Editors, American Journal of Hematology, Winship Cancer Institute, Emory University, 1365-B Clifton Road, Suite B4100, Atlanta, GA 30322 to permit rapid consideration for publication.

A Singular Case of Multiple Myeloma and Primary Biliary Cirrhosis Strictly Associated in Pathogenesis and Response to Alkylating Therapy To the Editor: Primary biliary cirrhosis (PBC) is a chronic and progressive cholestatic liver disease. The etiology is unknown, although it is presumed to be autoimmune. The data supporting the autoimmune origin of PBC are as follows [1,2]: abnormalities of the humoral and cellular immune systems (i.e., elevated serum levels of immunoglobulins, IgM in 80–90% of cases), multiple circulating autoantibodies, an impaired regulation of both B and T lymphocytes, and the association with a variety of autoimmune-mediated diseases such as systemic lupus erythematosus, autoimmune thyroiditis, Raynaud syndrome, Sjo¨gren syndrome, and scleroderma. Detection of IgG anti-mitochondrial antibodies (AMA) is present in over 90% of patients. In this report we illustrate an unusual association between PBC and multiple myeloma (MM) with a very good response to alkylating agent therapy. A 59-year-old female was referred to our department with recent diagnosis of PBC, occasionally detected on the basis of circulating autoantibodies (AMA and antinuclear) with elevated monoclonal component. Liver biopsy showed a stage III (Ludwig and coll) PBC, with ultrasonography evidence of splenomegaly (20 cm diameter) without signs of extrahepatic biliary tract obstruction. Laboratory tests were negative for hepatitis B and C viruses. Bone marrow biopsy with laboratory and radiologic findings was diagnostic for MM IgG, Bence-Jones positive, stage IA, with mild pancytopenia. Immunological findings showed positive ANA (titer 1:2400), AMA, and rheumatoid factor and positive Waaler Rose reaction (titer 1:20). As a consequence of high monoclonal component and by suggestion of the association between the plasma cell disorder and the altered immune function with hepatic involvement, the patient was treated for hematologic disease. She received alkylating chemotherapy with melphalan (10 mg/day for 4 days every 6 weeks) and methylprednisolone (50 mg/day for 4 days every 6 weeks) for eight cycles. The treatment was well tolerated without toxicities. Laboratory results after eight cycles of therapy showed a consensual liver and immunologic and hematologic response. All altered parameters showed significant improvement: ANA titer decreased from 1:2400 to 1:600, AMA became negative, rheumatoid factor decreased from 59.9 to 46 UI/ml, and Waaler Rose reaction became negative. Serum alkaline phosphatase was lower (646 to 429 UI/ml), while the C 2006 Wiley-Liss, Inc. V

transaminase level remained normal. Coagulation laboratory findings connected with impaired liver function became normal (prothrombine activity from 62 to 78%, activated partial thromboplastin time from 36.8 to 33.7 s). The monoclonal component was significantly lower (from 3.2 to 1.6 g/dl, IgG from 3.412 to 2.088 mg/dl); the 2-microglobulin level decreased from 9.14 to 4.14 mg/L, and BenceJones became negative (45 mg/dl before therapy). The IgM level decreased (458 to 219 mg/dl). The response to therapy is still maintained after a 2-year negative follow up. Although this association is rare with few reports in the literature [3–5], this case confirms the pathogenetic role of immuneregulatory abnormalities in PBC. The correlation between PBC and MM is demonstrated by the consensual response to alkylating therapy. This report can be a useful observation to suggest the involvement of immune response in PBC and represents a new association of PBC with a hematologic disease strictly connected to the immune system as MM.

ANTONIO LAZZARO PATRIZIA BERNUZZI ANNALISA ARCARI RAFFAELLA BERTE` FILIPPO CARLO MORONI ELENA TRABACCHI DANIELE VALLISA LUIGI CAVANNA Department of Clinical Oncology and Hematology, Hospital Gulielmo da Saliceto, Piacenza, Italy Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/ajh.20586

REFERENCES 1. Gershwin ME, Selmi C, Worman HJ, Gold EB, Watnik M, Utts J, Lindor KD, Kaplan MM, Vierling JM. Risk factors and comorbidities in primary biliary cirrhosis: a controlled interview-based study of 1032 patients. Hepatology 2005;42:1194–1202. 2. Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med 2005;353:1261– 1273. 3. Rodriguez-Leal GA, Moran-Villota S, Arista-Nasr J, Uribe-Esquivel M. Case report of multiple myeloma and hypothyroidism in primary biliary cirrhosis. Rev Invest Clin 1997;49:215–220. 4. Kaneko H, Endo T, Saitoh H, Katsuta Y, Aramaki T, Hayakawa H. Primary biliary cirrhosis associated with multiple myeloma. Intern Med 1993;32:802–805. 5. Akashi Y, Yoshizawa N, Kubota T, Oshikawa Y, Oda T, Ishida A, Nakabayashi I, Nishiyama J, Tazawa K. Primary biliary cirrhosis complicated with Sjogren syndrome and multiple myeloma. A case report. Nephron 1996;73:730–732.

Myelodysplasia with Erythroid Hypoplasia To the Editor: Various hematological malignancies have been associated with severe erythroid hypoplasia, especially chronic lymphocytic leukemias, lymphomas, both Hodgkin’s and non Hodgkin’s, and Waldernstrom’s macroglobulinemias. However, the association of myelodysplasia (MDS) with erythroid hypoplasia is a very rare disorder. The morphological and clinical features of this

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Letters and Correspondence may be regarded as a distinct clinicopathological entity and its association with specific chromosomal defects, treatment protocols, and final outcome be identified.

S. RAWAT R. THAKUR Department of Haematology–Clinical Pathology, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi 110044, India Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/ajh.20660

REFERENCES

Fig. 1. Bone marrow biopsy section showing a cluster of blasts along with mature myeloid cells, eosinophils, and plasma cells. There is a paucity of erythroid cells. An abnormal mitotic figure is also present. Hematoxylin and eosin 400. [Color figure can be viewed in the online issue, which is available at www.interscience.wiley.com.]

have thus far not been clearly defined [1,2] and in most patients it is mistaken for acquired pure red cell aplasia. Review of the literature reveals very few reports of this entity [3]. In a series of 360 cases of myelodysplasia diagnosed in a single institute Williamson et al. [4] reported 6 cases of myelodysplasia with red cell hypoplasia. Garcia Suarez et al. [5], came across only 16 well-documented cases, including their 1 case, in an extensive review of the literature. Recently Goyal et al. [1] reported 3 cases in the pediatric age group. We report a case of MDS with erythroid hypoplasia with increased plasma cell infiltrate in an elderly transfusion-dependent male. A 61-year-old male presented with weakness and breathlessness of 2 months’ duration. He had been found to be severely anemic and had received several courses of hematinics and multiple blood transfusions. The patient had severe pallor and grade III dyspnea. There was no lymphadenopathy or organomegaly. Hemoglobin concentration was 4 gm/dl and reticulocyte count was less than 0.1%. Total leukocyte and platelet counts were within normal limits. Bone marrow aspirate revealed marked erythroid depression and the presence of 4–5 % blasts. There were features of dysmyelopoiesis, dysgranulopoiesis, and dysmegakaryopoiesis. Plasma cells were increased. Biopsy sections revealed a hypercellular marrow with disarray of architecture. There was paucity of erythroid cells. Very occasional glycophorin-positive cells were present. Small clusters of immature cells were present in abnormal locations. They were positive for LCA and negative for CD3, CD20, and glycophorin. Dysmyelopoiesis, dysmegakaryopoiesis, and increased reticulin fibrosis were also noted. There was increase in eosinophils and its precursors in the marrow (see Figure 1). A notable feature was dense infiltration of the marrow by plasma cells and lymphocytes. The lymphocytic infiltrate showed an admixture of CD3-positive lymphocytes and CD20-positive lymphocytes. Serum electrophoresis was suggestive of polyclonal hypergammaglobulinemia. MDS characteristically exhibits a high number of erythroid precursors in marrow. In our case, the presence of immature cells in abnormal locations, dysmyelopoiesis, and dysmegakaryopoiesis associated with erythroid hypoplasia supported the diagnosis of myelodysplasia with red cell hypoplasia. The mechanism of erythroid hypoplasia in MDS is unknown. Various workers have postulated stem cell defect [5], whereas others have pointed to an autoimmune pathology [2]. The findings of plasma cells and lymphocytes in our case renders credence to an autoimmune pathology. The prognostic significance of MDS with erythroid hypoplasia is premature due to the small number of cases reported in the literature. It is suggested that it

American Journal of Hematology DOI 10.1002/ajh

1. Goyal R, Varma N, Marwah RK. Myelodysplastic syndrome with erythroid hypoplasia. J Clin Pathol 2005;58:320. 2. Kim HD, Kim HW, Park Y, et al. Myelodysplastic syndrome with erythroid aplasia following pure red cell aplasia. Korean J Intern Med 2004;19:193. 3. Cook MK. Red cell hypoplasia associated with myeloproliferative and myelodysplastic syndrome. J Clin Pathol 1989;42:890. 4. Williamson PJ, Oscier DJ, Bell AJ, et al. Red cell aplasia in myelodysplastic syndrome. J Clin Pathol 1991;44:431. 5. Garcia Suarez J, Pascual T, Munoz MA, et al. Myelodysplastic syndrome with erythroid hypoplasia/aplasia: a case report and review of the literature. Am J Haematol 1998;58:319.

Clinically Significant Pseudohyponatremia To the Editor: The term pseudohyponatremia may inadvertently connote a clinically unimportant laboratory artifact without clinical significance for patients. It occurs due to a shift of measurable sodium in the aqueous phase of a plasma sample following infusion of immune globulins, among other causes [1]. Steinberger et al. reported a prospective observational study of 18 IVIG infusions given at 2 g/kg administered over 2 to 5 days causing significant increases in serum protein, viscosity, and osmolar gap, with decreases of serum sodium and calculated osmolality without associated symptoms from the hyponatremia [2]. Most complications from pseudohyponatremia have been associated with subsequent medical interventions that were unnecessarily applied, such as diuresis, fluid restrictions, and giving hypertonic saline, thus making it important to distinguish true hyponatremia from pseudohyponatremia [3]. This case report indicates that transient pseudohyponatremia may be clinically important for critical target organs. A 72-year-old female with multiple sclerosis received 400 mg/kg monthly IVIG infused at 0.2 mg/kg per min for 30 mins, and when tolerated the infusion was increased to 0.3 mg/kg per min for 30 min and finally to 0.4 mg/kg per min until completion. The first 9 courses were uncomplicated with the durations of infusion between 2 h 30 min and 3 h 45 min. Following the 10th infusion, given over 3 h 15 mins, she had acute and dramatic worsening of neurologic symptoms. She was suddenly unable to bear weight or coordinate arm and leg movements and described paresthesias and hyposthesias of all extremities. Blood chemistries were normal except for the serum sodium of 130 mEq/dl. The new symptoms cleared without therapeutic intervention after 45 min. At her next visit the baseline sodium was 138 mEq/dl. After the same amount of immunoglobulin was given over 3 h 45 min the serum sodium fell to 131 mEq/dl. The serum osmolality increased from 286 to 294 mOsmol/kg (normal: 280–300 mOsmol/kg). It appears, therefore, that this patient’s precarious neurologic situation suddenly worsened related to the acute hyponatremia. Slowing the subsequent infusions did not prevent the hyponatremia but did prevent the acute worsening of symptoms. This letter is to warn that the appearance of pseudohyponatremia can be clinically significant in patients at risk. We hypothesize that her transient neurological worsening was due to water shifts into already dysfunctional cells. That it occurred following one session and not others may be a reflection of

Letters and Correspondence other shifts in total body water of which we were unaware. Colls reported similar falls in serum sodium among patients with Guillain–Barre´ syndrome receiving IVIG, following which several of the older and more ill died [4]. In cases with such vulnerable targets the infusions need be slowed to allow a more gentle accommodation of fluid shifts.

MURRAY BERN Cancer Center of Boston and Department of Medicine, Harvard Medical School, Boston, Massachusetts Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/ajh.20645

REFERENCES 1. Koffman BM, Dalakas MC. Effect of high-dose intravenous immunoglobulin on serum chemistry, hematology, and lymphocyte subpopulations: assessments based on controlled treatment trails in patients with neurological disease. Muscle Nerve 1997;20:1102–1107. 2. Steinberger BA, Ford SM, Coleman TA. Intravenous immunoglobulin therapy in postinfusional hyperproteinemia, increased serum viscosity, and pseudohyponatremia. Am J Hematol. 2003;73:97–100. 3. Steinberger B, Coleman TA. Multiple complications of IVIG therapy in a patient with Guillain–Barre syndrome. Am J Hematol 2001;67:59. 4. Colls BM. Guillain–Barre syndrome and hyponatremia. Intern Med J 2003;33:5–9.

Idiopathic Myelofibrosis Associated with Dermatomyositis To the Editor: Chronic idiopathic myelofibrosis (IM) is characterized by splenomegaly, immature peripheral blood granulocytes, and erythrocytes, with teardrop-shape red cells [1]. Several kinds of autoimmune disease have been reported in patients of IM [2–4]. However, to the best of our knowledge there was no previous case of dermatomyositis (DM) reported in a patient of chronic IM.

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A 62-year-old female was admitted for evaluation of chronic anemia. The patient complained of worsening dysphagia, weakness, and pain of the proximal upper and lower extremity muscles of 2 months’ duration. Skin involvement including Gottron’s papule, heliotrope rash, and periungual telangiectasis was observed, with decrease in the strength of the shoulder and the hip girdle muscles bilaterally. The abdomen exam showed splenomegaly. Further workup revealed hemoglobin (8.2 gm/dl), hematocrit (24.9%), reticulocyte count (2.1%), and platelet count (128 109/L), with elevation of aspartate aminotransferase (82 U/L), alanine aminotransferase (53 U/L), serum creatine kinase (622 U/L), lactate dehydrogenase (469 U/L), aldolase (17.4 U/L), and serum myoglobin (741 /L). Peripheral smear showed teardrop cells with moderate anisocytosis and polychromia. CT scan of the abdomen confirmed splenomegaly. The electroneuromyogram showed fibrillation potentials with positive sharp waves in the deltoid and tensor fascia lata consistent with inflammatory myopathy, polymyositis, or DM. The patient underwent a deltoid muscle biopsy (Fig. 1) that confirmed the diagnosis of DM. Bone marrow aspiration was a dry tap. A core biopsy revealed a hypercellular marrow for age with increased megakaryocytes, clustering of megakaryocytes, and increased marrow reticulin, with fibrosis, most consistent with the cellular phase of myelofibrosis. No visceral malignancies were detected. The patient was started on steroids (40 mg prednisone daily) that resulted in the improvement of skin lesions, anemia, muscle strength, and dysphagia over a period of 1 month. Our patient was diagnosed as having IM (on the basis of the hematologic findings with splenomegaly and confirmed by the bone marrow biopsy), and she was also diagnosed as having dermatomyositis. Several types of autoimmune disease such as SLE, rheumatoid arthritis, Sjogren’s syndrome, vasculitis, autoimmune hemolytic anemia, Sweet syndrome, idiopathic thrombocytopenic purpura, Hashimoto thyroiditis, and polyarteritis nodosa have been reported in patients of IM [2–4]. The incidence of the coexistence of autoimmune diseases is reported to be 10–14% [3,4]. Immune complexes are detectable in a high percentage of patients with IM. Their presence is associated with evidence of bone marrow histological markers of immune activity, and IgG was the main immunoglobulin class associated with IM [5].

Fig. 1. Deltoid muscle biopsy with perivascular lymphocytes, characteristic of dermatomyositis. [Color figure can be viewed in the online issue, which is available at www.interscience.wiley.com.]


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The present case represents a new association of idiopathic myelofibrosis with an autoimmune disease and gives support to the hypothesis of a possible immune basis of some IM cases. We believe that evidence of IM should be sought in any patient with autoimmune disease when associated with extramedullary hematopoiesis and hematological picture suggesting IM.

ALAA MUSLIMANI1 MANMEET S. AHLUWALIA1 POORNANAND PALAPARTY2 HAMED A. DAW2 1

Department of Medicine, Fairview Hospital, Cleveland Clinic Health System, Cleveland, Ohio 2 Moll Cancer Center, Cleveland Clinic Health System, Cleveland, Ohio Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/ajh.20594

REFERENCES 1. Tefferi A. Myelofibrosis with myeloid metaplasia. N Engl J Med 2000;342:1255. 2. Castro M, Conn DL, Su WP, Garton JP. Rheumatic manifestations in myelodysplastic syndromes. J Rheumatol 1991;18:721–727. 3. Enright H, Miller W. Autoimmune phenomena in patients with myelodysplastic syndromes. Leuk Lymphoma 1997;24:483–489. 4. Okamoto T, Okada M, Mori A, Saheki K, Takatsuka H, Wada H, Tamura A, Fujimori Y, Takemoto Y, Kanamaru A, Kakishita E. Correlation between immunological abnormalities and prognosis in myelodysplastic syndrome patients. Int J Hematol 1997;66:345–351. 5. Cappio FC, Vigiliani R, Novarino, Camussi G, campana D, Gavosoyo F. Idiopathic myelofibrosis : a possiple role for immune-complexes in the pathogenesis of bone marrow fibrosis. Br J Haematol 1981;49:17–21.

Development of Secondary Myelodysplastic Syndrome in a Patient with Previous Bladder Carcinoma To the Editor: Transitional cell carcinoma of the bladder was diagnosed in a 65-year-old male patient in 1998. The patient had a grade III, T2 carcinoma. Radical cystectomy, ureterectomy. and ileal conduit operations were performed. The patient did not receive chemotherapy or radiotherapy except surgery. On regular control visits, the patient was evaluated as being in remission. Myelodysplastic syndrome (MDS) was diagnosed 7 years after diagnosis and surgery of the bladder carcinoma while the patient was in remission. MDS was classified as RCMD and IPSS score was intermediate-1 according to WHO classification [1,2]. The patient had neutropenia, anemia, and thrombocytopenia at the time of MDS diagnosis and blast percentage in the bone marrow was 3.5%. Cytogenetic examination revealed 46XY karyotype. Transfusions of erythrocyte suspensions when needed were planned as first-line treatment because the EPO level was >200 mU/ml (9–30 mU/ml). The patient needed no platelet transfusions while his platelet values ranged between 50 and 100 109 L 1. The patient has a progressive disease with increasing erythrocyte transfusion requirement and low-dose thalidomide treatment was planned as salvage therapy. To our knowledge, secondary MDS has not been reported in the literature after bladder carcinoma. There is a report of two cases of AML after urothelial cancer, which had been treated with chemotherapy and radiotherapy [3]. Chemotherapy regimens consisting of drugs that might affect bone marrow directly could cause secondary hematologic malignancies and also radiotherapy applied to the sites in which major hemopoetic tissues are located could be the underlying mechanism of secondary hematologic malignancies [4]. Both of these mechanisms could be implicated in both of these reported patients. Although secondary or therapy-related hematologic malignancies, mainly chemotherapyinduced leukemias and myelodysplasias, represent possible complications of anticancer treatments, in our patient the development of MDS is not related to


previous cytotoxic therapies. Although the disease development can be just a coexistence when the age of the patient is considered along with the high incidence of MDS in this age and sex group, the underlying primary malignancy could play a substantial role in MDS development. The time interval between primary bladder carcinoma and the onset of MDS suggests that the disease is secondary. In our case, successful treatment of bladder carcinoma with only surgical intervention allowed the patient to survive long enough to develop MDS. The presence of a previous malignant condition makes the patient more susceptible to developing another malignant, or likewise in our case, a premalignant condition. Since anemia or other cytopenias are frequently seen in patients having hematological malignancy, our case reminds us that the probability of development of MDS should be kept in mind and should be carefully examined in patients with refractory anemia or cytopenia.

AHMET IFRAN1 ORAL NEVRUZ1 FERIT AVCU1 S¸EFIK GU¨RAN2 ALI UG˘UR URAL1 1 Department of Hematology and 2Department of Genetics, Gu¨lhane Military Medical Academy, 06010 Etlik, Ankara, Turkey Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/ajh.20630

REFERENCES 1. Wardiman JW, Harris NL, Brunning RD. The World Health Organization (WHO) classification of the myeloid neoplasms. Blood 2002;100:2292–2302. 2. Greenberg P, Cox C, LeBeau MM, et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood 1997;89:2079–2088. 3. Theodore C, Bayle A, Wibault P. Secondary leukaemia after treating advanced bladder cancer with methotrexate, vinblastine, doxorubicin and cisplatin chemotherapy and radiotherapy. BJU Int 2002;90:470–471. 4. Leone G, Voso MT, Sica S, et al. Therapy related leukemias: susceptibility, prevention and treatment. Leuk Lymphoma 2001;41:255–276.

Implantable Cardioverter Defibrillator Therapy in a Patient with Cardiac Amyloidosis To the Editor: Light-chain associated (AL) amyloidosis is a systemic disease characterized by clonal plasma cell dyscrasia and extracellular deposition of protein with light-chain fragments [1], which causes severe organ failure due to amyloid deposition to systemic organs. Cardiac amyloidosis causes arrhythmia, conduction disturbance, and congestive heart failure (CHF), which can be fatal. For amyloidosis with CHF, the median survival is 4 months. Effective treatment for AL amyloidosis has not yet been found. High-dose melphalan with autologous peripheral blood stem cell transplantation (PBSCT) has been tried with selected AL amyloidosis patients [2], but most patients have multisystem organ problems and they cannot undergo transplantation because of the high risk. However, an implantable cardioverter defibrillator (ICD) has recently been used as a new strategy to deal with fatal ventricular tachycardia, which occurs with such conditions as myocardosis and Brugada’s syndrome, and several reports have described the availability of this device [3]. Here we report a case of progressive cardiac amyloidosis in which an ICD prolonged the patient’s life for a certain period. A 52-year-old Japanese male was diagnosed with multiple myeloma in March 2004. His serum IgA level was 137 mg/dl. His M-protein was of IgA and Bence Jones protein types. Bone marrow aspiration disclosed an increase in plasma cells (26.6%). Ulcers of the tongue, anemia (hemoglobin 12.3 g/dl), hypercalcemia (serum calcium 10.3 mg/dl), proteinuria (urine protein 1,170 mg/day), and carpal tunnel syndrome were observed. Tongue and gastric biopsies revealed amyloid deposition, and a diagnosis of AL amyloidosis was made. He was

Letters and Correspondence treated with vincristine, doxorubicin, and dexamethasone, but with no effect. Autologous PBSCT was planned, but on July 2, he experienced a temporary loss of consciousness due to ventricular tachycardia. Normal heart function was regained with a cardiac defibrillator. Suspected amyloid deposition in the myocardium was demonstrated by echocardiography. A cardiac biopsy confirmed cardiac amyloidosis. Autologous PBSCT was canceled because of the high risk. On July 26, implantation of an ICD was carried out because we believed that possibly fatal arrhythmia could occur again. During 2 months after implantation, he experienced five episodes of possibly fatal ventricular tachycardia. The ICD worked precisely every time, and he was saved. Finally, on October 2, he died of multiple organ failure, but CHF and arrythmia did not cause his death. AL amyloidosis is a progressive disease. In this case, it was extremely aggressive and the patient finally died. However, implantation of the ICD did have an effect on his cardiac amyloidosis. Although some cardiologists consider that ICD may be indicated in patients with cardiac amyloidosis, implantation is difficult because of the poor prognosis for amyloidosis patients [4]. There have been only a few reports regarding the placement of an ICD in cases of cardiac amyloidosis [5]. To the best of our knowledge, this is the first report of case in which an ICD had some effect, although that effect was limited in this case. We believe that in some cases, however, an ICD may improve the prognosis for cardiac amyloidosis to a certain extent.

MITSURU ITOH1 KONOSUKE OHMORI2 KENICHIRO YATA2 HIDEHO WADA2 TETSUO TSUCHIYA3

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NORIKO OKAHASHI3 SOHEI HAMANAKA4 KIYOSHI YOSHIDA3 KAZUO TANEMOTO4 TAKASHI SUGIHARA2 1

Division of Clinical Pathology and Laboratory Medicine, Division of Hematology, Department of Medicine, 3 Division of Cardiovascular Diseases, Department of Medicine, and 4 Department of Thoracic and Cardiovascular Surgery, Kawasaki Medical School, Kurashiki, 701–0192, Japan Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/ajh.20631 2

REFERENCES 1. Gillmore JD, Hawkins PN, Pepys MB. Amyloidosis: a review of recent diagnostic and therapeutic developments. Br J Haematol 1997;99:245–256. 2. Comenzo RL, Gertz MA. Autologous stem cell transplantation for primary systemic amyloidosis. Blood 2002;99:4276–4282. 3. Moss AJ, Hall WJ, Cannom DS, Daubert JP, Higgins SL, Klein H, Levine JH, Saksena S, Waldo AL, Wilber D, Brown MW, Heo M. Improved survival with an implanted defibrillator in patients with coronary disease at high risk for ventricular arrhythmia. N Engl J Med 1996;335:1933–1940. 4. Kothari SS, Ramakrishnan S, Bahl VK. Cardiac amyloidosis—an update. Indian Heart J 2004;56:197–203. 5. Hess EP, White RD. Out-of-hospital cardiac arrest in patients with cardiac amyloidosis: presenting rhythms, management and outcomes in four patients. Resuscitation 2004;60: 105–111.
