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Eosinophilia with Organ Involvement in 3 Siblings Jpn. J. Clin. Immunol., 35 (6) 533～538 (2012)2012 The Japan Society for Clinical Immunology 533533 Case Report Eosinophilia with organ involvement in 3 siblings Mineto OTA1, Kenchi TAKENAKA1,MafuyuTAKAHASHI2 and Kenji NAGASAKA1 1Department of Rheumatology, Ome Municipal General Hospital 2Department of Neurology, Ome Municipal General Hospital (Received June 11, 2012) summary We describe 3 siblings who suŠered from marked eosinophilia with organ involvement. One sibling, who ex- perienced cervical lymphadenopathy and peripheral neuropathy with eosinophilia (5,834 cells/mL) following bronchial asthma, was diagnosed with Churg-Strauss syndrome (CSS) according to the criteria of the American College of Rheumatology. Another sibling, who suŠered from severe asthma with persistent polyarthritis and eosinophilia (2,496 cells/mL), was also diagnosed with CSS according to the criteria of the Japanese Ministry of Health, Labour and Wel- fare. The remaining sibling, who had eosinophilic pleuritis with peripheral blood eosinophilia (699 cells/mL), did not fulˆll the widely used criteria for CSS or hypereosinophilic syndrome (HES) ; however, he ˆt the newly proposed criteria for HES. Glucocorticoid treatment relieved their symptoms. Although the diagnoses and the criteria used for diagnosis diŠered between the siblings, all 3 patients showed common features such as eosinophilia with organ involve- ment that required treatment, indicating the possibility of familial eosinophilia (FE).Furthermore,theclinicalfea- tures observed diŠered substantially from those of previously reported FE patients, therefore, these 3 siblings may be aŠected by a type of FE distinguishable from those previously described. Key words―Churg-Strauss syndrome; hypereosinophilic syndrome; familial eosinophilia and Welfare9). The other sibling, who had pleuritis Introduction accompanied by eosinophil inˆltration, did not fulˆll Eosinophilia may be caused by several factors, in- the advocated criteria for CSS8～10) or HES11,12). All 3 cluding allergic reactions, parasitic infections, and siblings required treatment with glucocorticoids malignancy. Churg-Strauss syndrome (CSS) and (GC). hypereosinophilic syndrome (HES), which are rare Although their diagnoses and the criteria used for disorders with an unknown etiology, are known to be diagnosis diŠered between the siblings, these 3 accompanied by peripheral blood eosinophilia. patients showed common features such as eo- However, they are clearly distinguished from allergic sinophilia with organ involvement requiring treat- reactions because they have a distinctive feature, ment. Because CSS and HES are both uncommon namely, organ damage1,2). disorders, we believe that these ˆndings cannot be Many reports have described familial eosinophilia merely coincidental. Furthermore, the clinical fea- (FE) since the early 1900 s3,4). In these families, the tures observed diŠered substantially from those of distribution of eosinophilia was suggested to involve previously reported FE patients, therefore, these 3 si- autosomal dominant inheritance. Recently, another blings may be aŠected by a type of FE distinguisha- autosomal dominant type of FE has also been ble from those previously described. described5～7). The disorder was characterized not Case report only by marked eosinophilia but also by progression to end-organ damage in some aŠected family mem- Case 1 : The sixth sibling (Fig. 1) bers, similar to HES. Furthermore, some of these A 42-year-old female housekeeper acquired bron- family members exhibited obstructive pulmonary dis- chial asthma in 2001. She was a non-smoker. In easessuchasasthma. 2002, she noticed enlargement of the cervical lymph We encountered 3 siblings who suŠered from nodes and pruritus on her body. She then ex- marked eosinophilia with organ involvement. One si- perienced paralysis of both her hands. Later, she was bling fulˆlled the criteria of the American College of conˆnedtoherbedandwasthenadmittedtoour Rheumatology (ACR) for CSS8), whereas another hospital. Physical examination showed severe distal did not fulˆll the ACR criteria for CSS but did meet upper limb weakness, paresthesia in the right upper those of the Japanese Ministry of Health, Labour and lower limbs, and eruptions on her trunk and ex- 534 日本臨床免疫学会会誌 (Vol. 35 No. 6) Figure 1. Family pedigree Of 6 siblings, 3 had eosinophilia with organ involvement (Cases 13). The 3 siblings without eosinophilia did not have a history of asthma. Only the oldest sister had a skin disorder. As for their parents, children, grandparents, and parents' siblings, 5 had dermatologic abnormalities and 2 had a history of asthma, but none had medical conditions similar to those seen in Cases 13. tremities. Blood examination showed leukocytosis ‰uorescent in situ hybridization did not show a 4q12 (13,260 cells/mL, 44[5,834 cells/mL] of which deletion, indicating that the FIP1L1-platelet-derived were eosinophils). The C-reactive protein (CRP) lev- growth factor receptor fusion gene was absent. Her el was slightly elevated (1.49 mg/dL).SerumIgElev- IgE level returned to normal (98 IU/mL);however,a el was elevated (625 IU/mL);however,IgEspeciˆc positive level (0.90 UA/mL) of a speciˆc IgE against to multiple inhaled or ingested allergens was not de- moths was detected, without any related symptoms. tected by the CAP-RAST method. Myeloperoxidase Case 2 : The fourth sibling (Fig. 1) and proteinase-3 antineutrophil cytoplasmic antibo- A 43-year-old male car manufacturer with a history dies (MPO-ANCA and PR3-ANCA, respectively) of severe bronchial asthma since 1995 was admitted to were negative. A nerve conduction study showed a hospital due to worsening asthma, a persistent high mononeuropathy multiplex pattern. Neuromuscular fever (＞38°C) for 2 weeks, and outbreak of rash on and skin biopsy showed severe microvasculitis around the extremities and back in 2004. He had smoked 1 the peripheral nerves with eosinophil inˆltration. The pack of cigarettes daily for 22 years. Physical exami- bone marrow aspirate showed a slightly elevated eo- nation showed conjunctional injection, swelling with sinophil count (10); however, no atypical cells tenderness in the proximal interphalangeal and were seen. The cause of eosinophilia could not be metacarpophalangeal joints, and papulae on the ex- ascertained even after considering medication as a tremities and back (Fig. 2). Blood examination potential cause. She was diagnosed with CSS in accor- showed leukocytosis (11,400 cells/mL, 21.9[2,496 dance with the ACR criteria8). After treatment with cells/mL] of which were eosinophils). The CRP level 40 mg/day prednisolone, her peripheral blood eo- was elevated to 13.60 mg/dL. Serum IgE level was sinophil count decreased to 95 cells/mL, and she was normal (103 IU/mL). MPO-ANCA and PR3-ANCA able to walk again. Currently, she is being treated were negative. Stool assay did not show the presence with 5 mg/day prednisolone, and her condition of parasites. Abdominal and pelvic computed remains stable. Peripheral blood screening via tomography (CT) scan showed no enlarged lymph OTA・Eosinophilia with Organ Involvement in 3 Siblings 535 Figure 2. Skin ˆndings in Case 2. Papulae were seen on the extremities and back. Figure 3. Chest computed tomography ˆndings of patient 3. nodes or solid tumor. Upper gastrointestinal en- Chest CT showed right-sided pleural eŠusion. doscopy showed no apparent abnormalities. Bone marrow aspirate showed normocellular bone marrow, with an elevated eosinophil count (20.5) without ed a right pleural eŠusion (Fig. 3), following which atypical cells. No apparent etiology for eosinophilia, he was admitted to our hospital. Physical examina- including allergic reactions, parasitic infections, or tion showed prurigo on his trunk and extremities. malignancy including malignant lymphoma, could be Blood examination showed a normal leukocyte count determined. Although his symptoms did not fulˆll the of 6,030 cells/mL; however, the eosinophil count was ACR8) or Lanham's10) CSS criteria, he was diagnosed slightly elevated (699 cells/mL). The CRP level was withCSSinaccordancewiththecriteriaofthe slightly elevated (1.42 mg/dL). Serum IgE level was Japanese Ministry of Health, Labour and Welfare9). also elevated (986 IU/mL). MPO-ANCA was nega- He was treated with 30 mg/day prednisolone, which tive; however, the PR3-ANCA level was slightly at once relieved his asthma, high fever, joint swelling, elevated (4.7 U/mL). The pleural eŠusion contained and rash. Two months later, he suŠered from worsen- a number of leukocytes (3,567 cells/mL) and 60 of ing of asthma with severe breathing di‹culty; which were eosinophils. No atypical cells were found. however, additional high-dose GC therapy relieved Bone marrow aspirate showed normocellular bone the dyspnea. He is currently being treated with 10 marrow, with a slight elevation in eosinophils (7.1) mg/day prednisolone and is experiencing no dyspnea and no atypical cells. Peripheral blood screening us- or rash. Peripheral blood screening via ‰uorescent in ing the ‰uorescent in-situ hybridization method did situ hybridization did not show a 4q12 deletion. Se- not show 4q12 deletion. No obvious cause of eo- rum IgE level was still normal (39 IU/mL) and no sinophilia, other than occupation-related factors was speciˆc IgE was detected. noted. These features did not fulˆll the World Health Case 3 : The third sibling (Fig. 1) Organization classiˆcation for HES11,12) ;however, A 61-year-old male carpenter who did not have a they did fulˆll the recently
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