{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T04:17:03Z","timestamp":1791346623984,"version":"4.3.1"},"reference-count":26,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":5639,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1991,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The frequency and severity of the painful sickle cell crisis vary greatly among affected patients. Aside from a high level of Hb F(&gt;20%) there is no established parameter which may modulate the clinical severity of the disease. In this paper we describe two groups of adult patients with homozygous SS and present their characteristics. The division into these two groups was on the basis of relatively low RBC deformability (\u226437% of control) and high RBC deformability (&gt;65% of control) in the steady state. None of the patients had \u03b1\u2010gene deletion and all had Hb F level &lt;6.0%. Each patient was followed for a minimum of 3 years. The number of dense cells was quantitated by centrifugation on discontinuous Stractan gradient. RBC deformability index in isotonic medium (DI 290) was determined by ektacytometry and expressed as % of control. The patients with low RBC deformability had significantly less painful crises and more leg ulcers than those patients with high RBC deformability. The average number of dense cells was 22.2% and 9.8% of total circulating cells in the first and second group respectively. Moreover, the group with high red cell deformability had 33% mortality during the study period whereas no deaths occurred in the group with low RBC deformability.<\/jats:p><jats:p>The data indicate that there is a subset of patients with SS who have relatively few painful crises despite low Hb F level. We wish to designate these by the acronym MIDDD syndrome: <jats:italic>Mild<\/jats:italic> disease as far as painful crises are concerned, <jats:italic>I<\/jats:italic>ncreased number of <jats:italic>D<\/jats:italic>ense cells, and <jats:italic>D<\/jats:italic>ecreased red cell <jats:italic>D<\/jats:italic>eformability. In addition these patients have high incidence of leg ulcers, have low incidence of urinary tract infection, and less mortality. Cellular factors seem to contribute to the incidence of painful crises.<\/jats:p>","DOI":"10.1002\/ajh.2830360211","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T12:08:44Z","timestamp":1172059724000},"page":"122-130","source":"Crossref","is-referenced-by-count":84,"title":["Sickle cell anemia with few painful crises is characterized by decreased red cell deformability and increased number of dense cells"],"prefix":"10.1002","volume":"36","author":[{"given":"Samir K.","family":"Ballas","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198206173062402"},{"key":"e_1_2_1_3_2","doi-asserted-by":"crossref","first-page":"1353","DOI":"10.1182\/blood.V63.6.1353.1353","article-title":"Cooperative study of sickle cell disease: Effects of thalassemia and microcytosis on the hematologic and vasoocclusive severity of sickle cell anemia","volume":"63","author":"Steinberg MH","year":"1984","journal-title":"Blood"},{"key":"e_1_2_1_4_2","first-page":"65a","article-title":"The Senegalese subhaplotype results in decreased morbidity in patients with sickle cell anemia","volume":"68","author":"Powars D","year":"1986","journal-title":"Blood"},{"key":"e_1_2_1_5_2","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1182\/blood.V65.1.183.183","article-title":"Hemaglobin S polymer: Primary delerminent of the hemolytic and clinical severity of the sickling syndromes","volume":"65","author":"Brittenham GM","year":"1985","journal-title":"Blood"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V63.4.921.921"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V72.4.1216.1216"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V72.6.2056.2056"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1038\/1841877a0"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/5.2.563"},{"key":"e_1_2_1_11_2","doi-asserted-by":"crossref","first-page":"946","DOI":"10.1128\/jvi.15.4.946-953.1975","article-title":"Physical map of polyoma viral DNA fragments produced by cleavage with a restriction enzyme from Haemophilus aegyptius, endonuclease R. 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