Chronic eosinophilic leukemia with unique ... - Wiley Online Library

134 downloads 1816 Views 253KB Size Report
included if it is essential to support the communication. Letters not ... Center, University of South Florida, 12902 Magnolia Drive, Tampa,. FL 33612 to permit ...

American Journal of Hematology 68:301±303 (2001)

LETTERS AND CORRESPONDENCE Letters and correspondence submitted for possible publication must be identi®ed as such. Text length must not exceed 500 words and ®ve bibliographic references. A single concise ®gure 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 speci®cations will not be returned to authors. Letters to the Editor are utilized to communicate a single novel observation or ®nding. 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 Paul Chervenick, M.D., Editor of Brief Reports/Letters to Editors, American Journal of Hematology, H. Lee Mof®tt Cancer Center, University of South Florida, 12902 Magnolia Drive, Tampa, FL 33612 to permit rapid consideration for publication.

Chronic Eosinophilic Leukemia With Unique Chromosomal Abnormality, t(5;12) (q33;q22) To the Editor: Chronic eosinophilic leukemia (CEL) is a rare disease, and it is often dif®cult to differentiate CEL from other hypereosinophilic conditions such as parasite infections, allergic disorders, hypereosinophilic syndromes, and other leukemias (acute myelomonocytic leukemia with eosinophilia, chronic myelogenous leukemia). Chromosomal analysis is useful for diagnosis of CEL. We present a CEL case with a unique chromosomal abnormality. A 30-year-old man was referred to our hospital because of eosinophilia. On examination, mild systemic lymphoadenopathy was found. Hematological examination showed leukocytosis (34400/lL) and eosinophilia (7600/lL). Blood hemoglobin level and platelet count were within normal limit. Bone marrow aspiration specimen showed hypercellular marrow with eosinophilia (17.6%), but there was no excess of blasts. A chromosomal analysis of bone marrow cells showed a clonal abnormality of translocation, 46, XY, t(5;12) (q33;q22) (Fig. 1). A diagnosis of chronic eosinophilic leukemia was made by eosinophilia with the chromosomal abnormality. He received a steroid therapy and an alpha-interferon therapy, but these therapies were not e€ective. He underwent bone marrow transplantation from his sibling donor with conditioning of high dose cyclophosphamide and total body irradiation. He has remained in complete remission for more than 4 months. CEL is classi®ed as an atypical chronic myeloid disorder and no speci®c chromosomal abnormality has been reported [1]. However a fraction of CEL cases show cytogenetic abnormality involving a break in the q31-35 region of chromosome 5, where genes encording for IL-3, IL-5, GM-CSF and platelet-derived growth factor b receptor are located [2,3]. Bain reported that most frequent chromosomal abnormality was t(5;12) (q31;p13) [3]. The counterparts of 5q31-35 were reported as 12ql3, 1q23, 2p23, 9p33 and 16pl3 [3]. There were no reports of t(5;12) (q33;q22) in CEL.

ã 2001 Wiley-Liss, Inc.

Fig. 1. Chromosomal analysis of bone marrow cells. A clonal translocation, 46, XY, t(5;12) (q33;q22), was detected in all examined cells.

In our case, as the patient had the chromosomal translocation of the 5q31-5q35 region, it was postulated that these cytokine genes were relevant in pathogenesis of CEL [3]. On the other hand, there was Bcell translocation gene 1 (BTG1) in the 12q22 region [4]. However the role of BTG1 in CEL is unknown, it is postulated that BTG1 is relevant in activating 5q31-35 genes. CEL is a rare disease, and the etiology still remains unclear. Further research must be made for investigation of the mechanism of CEL.

ACKNOWLEDGMENT We thank the medical, nursing, and laboratory sta€ members of Hyogo Medical Center for Adults for their excellent cooperation. We are also grateful to SRL for chromosomal analysis. This study was partly supported by Fund of Cancer Research from Hyogo Total Health Association.

KIMIKAZU YAKUSHIJIN TOHRU MURAYAMA ISHIKAZU MIZUNO AKIKO SADA TAMIO KOIZUMI SHION IMOTO Hematology/Oncology Division, Department of Medicine, Hyogo Medical Center for Adults

REFERENCES 1. Tefferi A. Chronic myeloid disorders: classi®cation and treatment overview. Seminors in Hematology 2001;38:l 4. 2. Luciano L, Catalano L, Sarrantonio C, Guerriero A, Califano C, Rotoli B. a IFN-induced hematologic and cytogenetic remission in chronic eosinophilic leukemia with t(1;5). Haematologica 1999;84:651 653. 3. Bain BJ. Eosinophilic leukemias and the idiopathic hypereosinophilic syndrome. Br J Haematol 1996;95:2 9.


Letters and Correspondence

4. Rimokh R, Rounault JP, Wahbi K, Gadoux M, Lafage M, Archimbaud E, Charrin C, Gentilhomme O, Germain D, Samarut J, Maguad JP. A chromosome 12 coding region is juxtaposed to the MYC protooncogene locus in a t(8;12) (q24;q22) translocation in a case of B-cell chronic lymphocytic leukemia. Genes, Chromosomes & Cancer 1991;3:24 36.

Case Report of a Normal Hemoglobin at Presentation of Thrombotic Thrombocytopenic Purpura To the Editor: The ®rst case of Thrombotic Thrombocytopenic Purpura (TTP) was described in 1924 by Moschowitz [1]. TTP has been characterized by a pentad of clinical ®ndings, including microangiopathic hemolytic anemia, thrombocytopenia, renal disorders, fever, and ¯uctuating neurological signs. However, only 40% of patients present with this pentad of symptoms whereas 75% of patients present with the triad of microangiopathic hemolytic anemia, neurological changes, and thrombocytopenia. The annual incidence of TTP is 3.7 cases per 100,000. It is more common in females than in males (3:2), and has a peak incidence in the third decade of life. The mortality rate of TTP approaches 100% without treatment. With of plasmapheresis, long-term survival rates for TTP approach 90% [2]. We diagnosed a patient with TTP in July 2000, who presented with a normal hemoglobin of 13.2 g/dl. The patient had four ®ndings of the TTP pentad: thrombocytopenia, microangiopathic hemolysis, hematuria, and neurological changes presenting as headache and lapses of memory. The patient never developed a fever. The patient responded to plasma exchange treatment. The normal hemoglobin value at presentation led us to review the literature on hemoglobin values at presentation and diagnosis of TTP.

CASE The patient was a 34-year-old white female presenting with multiple bruises on face, arms, and legs for 2 weeks. She had a history of a left cerebral vascular accident (CVA) in 1994, and ®ve transient ischemic attacks (TIA), the most recent in January of 1998 when she was pregnant with her son. Two weeks prior while playing with her child, she noticed easy bruising. She was slow to respond that evening while talking on the phone. She also had some bright red blood in her stool twice over the two weeks, and one episode of blood tinged urine. She denied any epistaxis, bleeding gums, or vaginal bleeding. She denied fever, headache, dizziness, blurry vision, chills, nausea,

TABLE II. Other Clinical Laboratory Values on Admission Labs 7/24 Fibrinogen D-dimer ESR Hep C antib Hep BsAG Hep BsAB B-HCG VIIIVWF ANA

351 0.25 20 Neg Neg Neg Neg 98 1:80

vomiting, diarrhea, diaphoresis, shortness of breath, chest pain, abdominal pain, facial asymmetry, or focal weakness. Her past medical history of ischemic cerebral events was thoroughly evaluated at the time, but no arterial disease or increased coagulability was found. Protein C, S, Antithrombin III, homocysteine, factor V Leyden, and antiphospholipid antibodies were normal. Past medical history included hypertension treated with Procardia XL and Coumadin for stroke prophylaxis. She smokes less than one pack of cigarettes per day and is a recovered alcoholic. She has a history of recreational drug abuse, which she stopped ten years ago. She had intravenous drug abuse. Patient's mother has diabetes and father has rheumatoid arthritis. On physical exam she had a temperature of 99.9°F, a heart rate of 100/min, respirations of 16/min, and her blood pressure was 160/95. She had multiple petechiae and ecchymoses on the anterior and posterior surfaces of her arms, legs, and hands bilaterally. She had icteric sclera and an ecchymosis on her upper lip. No blood was present from her nares, and there was no lymphadenopathy, bruits, thyromegaly, or jugular venous distention (JVD). Her heart rate was slightly tachycardiac without any murmurs, rubs, or gallops. Lungs were clear and abdomen soft, nontender, and nondistended without organomegaly. Rectal exam was heme negative. Clubbing, cyanosis, and edema were not present. Neurological exam was normal. Labs on admission were: Hemoglobin of 13.2 g/dl, hematocrit of 37.5%, platelets 11,000/lL, and WBC 11,200/lL. MCV was 90fL, RDW 13. Di€erential consisted of 62% segs, 7 bands, 28 lymphocytes, and 3 monocytes. Liver function tests were: AST 26 U/L, ALT 13 U/ L, Alk Phos 104 U/L, Total Bilirubin 3.6 mg/dL, Direct 0.4 mg/dL, albumin 4.0 g/dL, LDH 752 U/L, amylase 90 U/L, and lipase 78 U/L. Her PT was 12.3 seconds, PTT 23 seconds. Peripheral smear showed many schistocytes, markedly decreased platelets, and normal WBC morphology. Chest X-ray was normal, the Urinanalysis results from 7/ 24 showed 100 mg/dl protein; sulfasal was 1‡, 1000 mg of glucose, large blood, nitrate positive, 11-20 RBC's, and 3-5 granular casts.

TABLE I. Clinical Laboratory Course

Hbg(g/dL) Plts(/lL) Retic% LDH(U/L) Bun/creat (mg/dL) Tbili (mg/dL) Plasma exchange

7/23 19:46

7/24 05:26

7/24 16:14

7/25 05:58

7/25 21:01

7/26 05:31

7/27 05:36

7/28 05:36

7/29 06:00

13.2 11

13.7 6 5.8 642 17/1.0

10.9 18

9.6 50 15/0.9

10.0 133 4.4 215 18/1.0

9.3 214

553 18/1.2

9.9 109 4.2 209 16/1.2

10.2 285 7.6 234

327 6.8 171

752 3.4


166 17/0.9

1.4 Yes


1.1 Yes




7/30 10:40


9.0 348 7.6

10.0 393 9.4

1.0 Yes

Letters and Correspondence Table I details our patient's clinical laboratory course from admission through her plasma exchange therapy. Table II gives other admitting labs prior to initiating plasma exchange therapy. Microangiopathic hemolytic anemia is characteristic of TTP with an average presenting hemoglobin of 7.8 g/dL in approximately 90% of cases. A review of the literature demonstrated only ten cases where the hemoglobin level was greater than 11 g/dL at presentation with the highest reported hemoglobin level being 14.0 g/dl [3]. Red blood cell indices are often normocytic normochromic, but the peripheral blood usually demonstrates numerous schistocytes and other misshapen red blood cells. Nucleated red blood cells may be present. Reticulocyte counts and LDH levels are normally elevated. The hemolytic anemia is Coombs' negative. Platelet counts are normally less than 20,000/lL. Hyperbilirubinemia is a common ®nding due to the increased RBC destruction. We report here the rare presentation of TTP without anemia, but with clear cut microangiopathic hemolysis and thrombocytopenia.


Initially, anemia may not be present until TTP evolves. We believe this case should remind us that microangiopathic hemolysis and thrombocytopenia should lead to a prompt consideration of TTP, regardless of the initial hemoglobin level.

DAVID H. HENRY MD HEATHER L. MARKOVITZ DO Pennsylvania Hospital, Philadelphia, Pennsylvania

REFERENCES 1. Moschowitz E. Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc NY Pathol Soc 1924;24:21. 2. Cuttner J. Thrombotic thrombocytopenic purpura: a ten year experience. Blood 1980;56:302. 3. Amorosi EL, Ultmann JE. Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature. Medicine 1966;45:139.

Suggest Documents