Immune thrombocytopenia (ITP) in children has a varied course and according to duration is distinguished as newly diagnosed (&lt;3 months), persistent (3–12), and chronic (&gt;12) types. Several studies have evaluated the prognostic factors for the progression of the disease, but similar works have yet to be performed in Greece. We aimed to identify prognostic markers for the three forms of the disease in 57 Greek children during a 13-year period. Information regarding age, gender, preceding infection, bleeding type, duration of symptoms and platelets at diagnosis, treatment, disease course, and immunological markers was recorded. 39 children had newly diagnosed, 4 persistent, and 14 chronic disease. Chronic ITP children were more likely to be of age &gt; 10 years <span class="inline_break"><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="23.471pt" style="vertical-align:-3.42947pt" id="M1" height="12.7178pt" version="1.1" viewBox="-0.0498162 -9.28833 23.471 12.7178"><g transform="matrix(.013,0,0,-0.013,0,0)"><path id="g113-41" d="M300 -147C201 -63 143 98 143 270S200 602 300 686L282 710C136 610 70 450 70 271V270C70 89 136 -72 282 -170L300 -147Z"/></g><g transform="matrix(.013,0,0,-0.013,4.498,0)"><path id="g113-113" d="M570 304C570 398 525 448 414 448C385 448 343 445 312 434L329 511L321 518C297 504 262 482 244 460L233 411C195 397 159 381 128 358L135 332C160 347 189 360 224 373L111 -147C97 -210 84 -218 17 -231L13 -257L254 -247L259 -218L233 -216C183 -212 177 -202 189 -142L218 -1C238 -10 266 -12 283 -12C351 3 429 48 483 105C543 168 570 242 570 304ZM482 289C482 161 380 33 304 33C278 33 248 51 233 69L303 396C326 400 352 403 369 403C428 403 482 380 482 289Z"/></g><g transform="matrix(.013,0,0,-0.013,15.84,0)"><path id="g117-34" d="M535 323V373H52V323H535ZM535 138V188H52V138H535Z"/></g></svg><span class="irelop"/><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="32.422pt" style="vertical-align:-3.42947pt" height="12.7178pt" version="1.1" viewBox="27.053183800000003 -9.28833 32.422 12.7178"><g transform="matrix(.013,0,0,-0.013,27.103,0)"><path id="g113-49" d="M241 635C89 635 35 457 35 312C35 153 89 -12 240 -12C390 -12 443 166 443 312C443 466 390 635 241 635ZM238 602C329 602 354 454 354 312C354 172 330 22 240 22C152 22 124 173 124 313S148 602 238 602Z"/></g><g transform="matrix(.013,0,0,-0.013,33.343,0)"><path id="g113-47" d="M113 -12C146 -12 170 11 170 46C170 78 146 103 114 103S58 78 58 46C58 11 82 -12 113 -12Z"/></g><g transform="matrix(.013,0,0,-0.013,36.307,0)"><use xlink:href="#g113-49"/></g><g transform="matrix(.013,0,0,-0.013,42.547,0)"><path id="g113-50" d="M384 0V27C293 34 287 42 287 114V635C232 613 172 594 109 583V559L157 557C201 555 205 550 205 499V114C205 42 199 34 109 27V0H384Z"/></g><g transform="matrix(.013,0,0,-0.013,48.787,0)"><path id="g113-54" d="M153 550H386L412 615L406 623H120L82 318C104 327 142 338 184 338C294 338 347 275 347 187C347 112 305 39 221 39C160 39 119 71 97 89C88 97 80 96 71 90C59 80 50 67 49 57C48 45 52 36 66 23C80 9 123 -12 169 -12C221 -11 288 15 342 59C403 109 431 165 431 225C431 308 366 395 238 395C212 395 165 379 127 364L153 550Z"/></g><g transform="matrix(.013,0,0,-0.013,55.027,0)"><path id="g113-42" d="M275 270C275 450 212 609 64 710L45 686C145 604 203 442 203 270S147 -63 45 -147L64 -170C213 -68 275 89 275 270Z"/></g></svg></span> and have gradual initiation of the disease <span class="inline_break"><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="23.471pt" style="vertical-align:-3.42947pt" id="M2" height="12.7178pt" version="1.1" viewBox="-0.0498162 -9.28833 23.471 12.7178"><g transform="matrix(.013,0,0,-0.013,0,0)"><use xlink:href="#g113-41"/></g><g transform="matrix(.013,0,0,-0.013,4.498,0)"><use xlink:href="#g113-113"/></g><g transform="matrix(.013,0,0,-0.013,15.84,0)"><use xlink:href="#g117-34"/></g></svg><span class="irelop"/><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="32.422pt" style="vertical-align:-3.42947pt" height="12.7178pt" version="1.1" viewBox="27.053183800000003 -9.28833 32.422 12.7178"><g transform="matrix(.013,0,0,-0.013,27.103,0)"><use xlink:href="#g113-49"/></g><g transform="matrix(.013,0,0,-0.013,33.343,0)"><use xlink:href="#g113-47"/></g><g transform="matrix(.013,0,0,-0.013,36.307,0)"><use xlink:href="#g113-49"/></g><g transform="matrix(.013,0,0,-0.013,42.547,0)"><use xlink:href="#g113-49"/></g><g transform="matrix(.013,0,0,-0.013,48.787,0)"><use xlink:href="#g113-50"/></g><g transform="matrix(.013,0,0,-0.013,55.027,0)"><use xlink:href="#g113-42"/></g></svg></span>, platelets &gt; 10 × 10<sup>9</sup>/L <span class="inline_break"><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="23.471pt" style="vertical-align:-3.42947pt" id="M3" height="12.7178pt" version="1.1" viewBox="-0.0498162 -9.28833 23.471 12.7178"><g transform="matrix(.013,0,0,-0.013,0,0)"><use xlink:href="#g113-41"/></g><g transform="matrix(.013,0,0,-0.013,4.498,0)"><use xlink:href="#g113-113"/></g><g transform="matrix(.013,0,0,-0.013,15.84,0)"><use xlink:href="#g117-34"/></g></svg><span class="irelop"/><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="26.182pt" style="vertical-align:-3.42947pt" height="12.7178pt" version="1.1" viewBox="27.053183800000003 -9.28833 26.182 12.7178"><g transform="matrix(.013,0,0,-0.013,27.103,0)"><use xlink:href="#g113-49"/></g><g transform="matrix(.013,0,0,-0.013,33.343,0)"><use xlink:href="#g113-47"/></g><g transform="matrix(.013,0,0,-0.013,36.307,0)"><use xlink:href="#g113-49"/></g><g transform="matrix(.013,0,0,-0.013,42.547,0)"><use xlink:href="#g113-50"/></g><g transform="matrix(.013,0,0,-0.013,48.787,0)"><use xlink:href="#g113-42"/></g></svg></span>, and impaired immunological markers <span class="inline_break"><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="23.471pt" style="vertical-align:-3.42947pt" id="M4" height="12.7178pt" version="1.1" viewBox="-0.0498162 -9.28833 23.471 12.7178"><g transform="matrix(.013,0,0,-0.013,0,0)"><use xlink:href="#g113-41"/></g><g transform="matrix(.013,0,0,-0.013,4.498,0)"><use xlink:href="#g113-113"/></g><g transform="matrix(.013,0,0,-0.013,15.84,0)"><path id="g117-91" d="M512 -3V55L134 254V256L512 456V514L75 281V230L512 -3Z"/></g></svg><span class="irelop"/><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="32.422pt" style="vertical-align:-3.42947pt" height="12.7178pt" version="1.1" viewBox="27.053183800000003 -9.28833 32.422 12.7178"><g transform="matrix(.013,0,0,-0.013,27.103,0)"><use xlink:href="#g113-49"/></g><g transform="matrix(.013,0,0,-0.013,33.343,0)"><use xlink:href="#g113-47"/></g><g transform="matrix(.013,0,0,-0.013,36.307,0)"><use xlink:href="#g113-49"/></g><g transform="matrix(.013,0,0,-0.013,42.547,0)"><use xlink:href="#g113-49"/></g><g transform="matrix(.013,0,0,-0.013,48.787,0)"><path id="g113-52" d="M285 378C315 398 338 416 353 432C373 451 384 474 384 503C384 579 325 635 236 635H235C182 635 136 610 108 579L65 516L85 496C110 533 150 575 205 575C258 575 300 543 300 481C300 407 232 369 141 339L147 310C163 315 188 321 211 321C268 321 338 284 338 192C338 94 288 40 217 40C160 40 119 68 93 91C85 98 77 97 69 91C60 84 47 71 46 58C44 46 48 35 62 22C75 10 116 -12 162 -12C234 -12 424 62 424 224C424 297 373 359 285 376V378Z"/></g><g transform="matrix(.013,0,0,-0.013,55.027,0)"><use xlink:href="#g113-42"/></g></svg></span> compared to newly diagnosed/persistent groups. Recent history of infection was found mainly in the newly diagnosed/persistent group <span class="inline_break"><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="23.471pt" style="vertical-align:-3.42947pt" id="M5" height="12.7178pt" version="1.1" viewBox="-0.0498162 -9.28833 23.471 12.7178"><g transform="matrix(.013,0,0,-0.013,0,0)"><use xlink:href="#g113-41"/></g><g transform="matrix(.013,0,0,-0.013,4.498,0)"><use xlink:href="#g113-113"/></g><g transform="matrix(.013,0,0,-0.013,15.84,0)"><use xlink:href="#g117-34"/></g></svg><span class="irelop"/><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="32.422pt" style="vertical-align:-3.42947pt" height="12.7178pt" version="1.1" viewBox="27.053183800000003 -9.28833 32.422 12.7178"><g transform="matrix(.013,0,0,-0.013,27.103,0)"><use xlink:href="#g113-49"/></g><g transform="matrix(.013,0,0,-0.013,33.343,0)"><use xlink:href="#g113-47"/></g><g transform="matrix(.013,0,0,-0.013,36.307,0)"><use xlink:href="#g113-49"/></g><g transform="matrix(.013,0,0,-0.013,42.547,0)"><use xlink:href="#g113-50"/></g><g transform="matrix(.013,0,0,-0.013,48.787,0)"><use xlink:href="#g113-52"/></g><g transform="matrix(.013,0,0,-0.013,55.027,0)"><use xlink:href="#g113-42"/></g></svg></span>. None of the children exhibited severe spontaneous bleeding.<i> Conclusion</i>. Even though ITP in children usually has a self-limited course, with rare serious bleeding complications, the chronic form of the disease is characterized by different predictive parameters, which can be used in clinical practice.

Alexandros Makis

Athanasios Gkoutsias

Theodoros Palianopoulos

Eleni Pappa

Evangelia Papapetrou

Christina Tsaousi

Eleftheria Hatzimichael

Nikolaos Chaliasos

Prognostic Factors for Immune Thrombocytopenia Outcome in Greek Children: A Retrospective Single-Centered Analysis

In the era of precision medicine, the impact of personalized dosing of busulfan is not clear. We undertook a retrospective analysis of 78 patients with myeloid malignancies who received fludarabine and busulfan (FluBu4) with or without measuring Bu pharmacokinetics (Bu PK) and those who received busulfan with cyclophosphamide (BuCy). Fifty-five patients received FluBu4, of whom 21 had Bu PK measured, and 23 patients received BuCy. Total donor cell chimerism showed that the percentage of patients maintaining 100&#x25; donor chimerism on day 100 was 66.7&#x25;, 38.2&#x25;, and 73.9&#x25; in the FluBu4 with PK, FluBu4 with no PK, and BuCy, respectively (<i>P</i> = .001). Patients who had decreasing donor chimerism by day 100 were 23.8&#x25;, 52.9&#x25;, and 26.1&#x25; in the FluBu4 with PK, FluBu4 with no PK, and BuCy, respectively (<i>P</i> = .04). Bu PK group had fewer patients with less than 95&#x25; donor chimerism on day 30, which was not statistically significant, 5&#x25; (FluBu4 PK), 31&#x25; (FluBu4 with no PK), and 21&#x25; (BuCy) (<i>P</i> = .18). Survival distributions were not statistically significant (<i>P</i> = .11). Thus, personalized drug dosing can impact donor chimerism in myeloid malignancies. This will need to be examined in larger retrospective multicenter studies and prospective clinical trials.

Shatha Farhan

Michael Bazydlo

Klodiana Neme

Nancy Mikulandric

Edward Peres

Nalini Janakiraman

Chimerism in Myeloid Malignancies following Stem Cell Transplantation Using FluBu4 with and without Busulfan Pharmacokinetics versus BuCy

Improved therapies are urgently needed for patients with diffuse large B cell lymphoma (DLBCL). Success using immune checkpoint inhibitors and chimeric antigen receptor T cell technology has fuelled demand for validated cancer epitopes. Immunogenic cancer testis antigens (CTAs), with their widespread expression in many tumours but highly restricted normal tissue distribution, represent attractive immunotherapeutic targets that may improve treatment options for DLBCL and other malignancies. Sperm protein 17 (Sp17), a CTA reported to be immunogenic in ovarian cancer and myeloma patients, is expressed in DLBCL. The aim of the present study was to investigate Sp17 epitope presentation via the presence of a cytotoxic T cell (CTL) and a CD4 T-helper (Th) response in DLBCL patients. A significant <i>γ</i>-interferon CTL response was detected in peripheral blood mononuclear cells of 13/31 DLBCL patients following short-term cell stimulation with two novel HLA-<svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" style="vertical-align:-0.04990005pt" id="M1" height="10.1524pt" version="1.1" viewBox="-0.0498162 -10.1025 15.1954 10.1524" width="15.1954pt"><g transform="matrix(.013,0,0,-0.013,0,0)"><path id="g121-63" d="M673 0V28C608 34 594 43 563 129C499 303 432 494 370 665L339 656L132 132C98 43 84 37 20 28V0H238V28C162 35 154 45 175 108C188 150 203 192 219 237H432C454 176 474 122 487 83S491 36 428 28V0H673ZM418 280H234C265 362 296 450 328 535H330L418 280Z"/></g><g transform="matrix(.0091,0,0,-0.0091,8.983,-5.741)"><path id="g50-43" d="M486 158C486 177 478 202 466 220C413 228 386 236 336 262C386 288 413 297 466 304C478 323 486 347 485 366C470 376 444 381 422 380C389 338 368 319 321 288C323 345 329 372 349 422C339 442 322 461 305 470C289 461 271 442 262 422C281 372 287 345 290 288C243 319 222 338 189 380C167 381 142 376 125 366C125 347 133 322 145 304C198 296 225 288 275 262C225 236 198 227 145 220C133 201 125 177 126 158C141 148 167 143 189 144C222 186 243 205 290 236C288 179 282 152 262 102C272 82 289 63 306 54C322 63 340 82 350 102C330 152 324 179 321 236C368 205 390 186 422 144C444 143 470 148 486 158Z"/></g></svg>0201 peptides and one previously reported HLA-<svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" style="vertical-align:-0.04990005pt" id="M2" height="10.1524pt" version="1.1" viewBox="-0.0498162 -10.1025 15.1954 10.1524" width="15.1954pt"><g transform="matrix(.013,0,0,-0.013,0,0)"><use xlink:href="#g121-63"/></g><g transform="matrix(.0091,0,0,-0.0091,8.983,-5.741)"><use xlink:href="#g50-43"/></g></svg>0101-restricted nine-mer Sp17 peptide. No significant responses were detected in the HLA-<svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" style="vertical-align:-0.04990005pt" id="M3" height="10.1524pt" version="1.1" viewBox="-0.0498162 -10.1025 15.1954 10.1524" width="15.1954pt"><g transform="matrix(.013,0,0,-0.013,0,0)"><use xlink:href="#g121-63"/></g><g transform="matrix(.0091,0,0,-0.0091,8.983,-5.741)"><use xlink:href="#g50-43"/></g></svg>0201-negative DLBCL patients or four healthy subjects. A novel immunogenic 20-mer CD4 Th Sp17 peptide was detected in 8/17 DLBCL patients. This is the first report of a CTL and a CD4 Th response to Sp17 in DLBCL and supports Sp17 as a potential immunotherapeutic target for DLBCL.

Kamel Ait-Tahar

Amanda P. Anderson

Martin Barnardo

Graham P. Collins

Chris S. R. Hatton

Alison H. Banham

Karen Pulford

Sp17 Protein Expression and Major Histocompatibility Class I and II Epitope Presentation in Diffuse Large B Cell Lymphoma Patients

The current study investigates the functional state of erythrocytes and indices of the oxygen-binding capacity of hemoglobin in blood samples from healthy donors and from patients with coronary artery disease and myocardial infarction before and after treatment. It has been established that, in cardiovascular diseases, erythrocyte morphology and hemoglobin oxygen-transporting disorders are observed. Standard therapy does not result in the restoration of the structure and properties of erythrocytes. The authors believe that it is necessary for future therapeutic treatment to include preparations other than cardiovascular agents to enhance the capacity of hemoglobin to transport oxygen to the tissues.

Victor V. Revin

Antonina A. Ushakova

Natalia V. Gromova

Larisa A. Balykova

Elvira S. Revina

Vera V. Stolyarova

Tatiana A. Stolbova

Ilya N. Solomadin

Alexander Yu. Tychkov

Nadezhda V. Revina

Oksana G. Imarova

Study of Erythrocyte Indices, Erythrocyte Morphometric Indicators, and Oxygen-Binding Properties of Hemoglobin Hematoporphyrin Patients with Cardiovascular Diseases

Relapsed/refractory acute myeloid leukemia (RR-AML) is associated with poor prognosis and long-term disease-free survival requires allogeneic hematopoietic cell transplantation (allo-HCT). Limited data exists, regarding the optimal regimen to obtain remission prior to allo-HCT. Single agent high-dose cytarabine (10&#x2013;12 doses administered every 12 hours) has been previously used as induction therapy. Six-dose high-dose cytarabine (HiDAC-6), commonly used as a consolidation regimen, has never been evaluated as induction therapy. We present a retrospective review of 26 consecutive patients with RR-AML receiving single agent cytarabine 3&#x2009;g/m<sup>2</sup> intravenously every 12 hours on days 1, 3, and 5 for a total of six doses (HiDAC-6). Median follow-up for surviving patients was 10.4 months (range 1.6&#x2013;112.2 months). Complete remission was obtained in 62&#x25; (54&#x25; CR and 8&#x25; CRi) of the patients. The median relapse-free survival (RFS) was 22.3 months (range 0.7&#x2013;112 months), event-free survival (EFS) was 4.7 months (range 0.5&#x2013;112 months), and the overall survival (OS) was 9.6 months (range 1&#x2013;112 months). Thirty-five percent of patients were able to subsequently proceed to allo-HCT. Treatment-related toxicities included neutropenic fever (38&#x25;), infection (35&#x25;), neurotoxicity (8&#x25;), and skin toxicity (8&#x25;). This is the first study to demonstrate HiDAC-6 as an active treatment option for younger patients with RR-AML which can effectively serve as a bridge to allo-HCT without significant toxicity.

Brandi Anders

Lauren Veltri

Abraham S. Kanate

Alexandra Shillingburg

Nilay Shah

Michael Craig

Aaron Cumpston

Outcomes of Six-Dose High-Dose Cytarabine as a Salvage Regimen for Patients with Relapsed/Refractory Acute Myeloid Leukemia

<i>Introduction</i>. The realization of red cell exchange (RCE) in Africa faces the lack of blood, transfusion safety, and equipment. We evaluated its efficacy and safety in severe complications of sickle cell disease.<i> Patients and Method</i>. Manual partial RCE was performed among sickle cell patients who had severe complications. Efficacy was evaluated by clinical evolution, blood count, and electrophoresis of hemoglobin. Safety was evaluated on adverse effects, infections, and alloimmunization.<i> Results</i>. We performed 166 partial RCE among 44 patients including 41 homozygous (SS) and 2 heterozygous composites SC and 1 S/<i>&#x3b2;</i>0-thalassemia. The mean age was 27.9 years. The sex ratio was 1.58. The regression of symptoms was complete in 100&#x25; of persistent vasoocclusive crisis and acute chest syndrome, 56.7&#x25; of intermittent priapism, and 30&#x25; of stroke. It was partial in 100&#x25; of leg ulcers and null in acute priapism. The mean variations of hemoglobin and hematocrit rate after one procedure were, respectively, +1.4&#x2009;g/dL and +4.4&#x25;. That of hemoglobin S after 2 consecutive RCE was &#x2212;60&#x25;. Neither alloimmunization nor viral seroconversion was observed.<i> Conclusion</i>. This work shows the feasibility of manual partial RCE in a low-resource setting and its efficacy and safety during complications of SCD outside of acute priapism.

B. F. Faye

D. Sow

M. Seck

N. Dieng

S. A. Toure

M. Gadji

A. B. Senghor

Y. B. Gueye

D. Sy

A. Sall

T. N. Dieye

A. O. Toure

S. Diop

Efficacy and Safety of Manual Partial Red Cell Exchange in the Management of Severe Complications of Sickle Cell Disease in a Developing Country

Multiple myeloma (MM) is a heterogeneous hematologic malignancy involving the proliferation of plasma cells derived by different genetic events contributing to the development, progression, and prognosis of this disease. Despite improvement in treatment strategies of MM over the last decade, the disease remains incurable. All efforts are currently focused on understanding the prognostic markers of the disease hoping to incorporate the new therapeutic modalities to convert the disease into curable one. We present this comprehensive review to summarize the current standard prognostic markers used in MM along with novel techniques that are still in development and highlight their implications in current clinical practice.

Amr Hanbali

Mona Hassanein

Walid Rasheed

Mahmoud Aljurf

Fahad Alsharif

The Evolution of Prognostic Factors in Multiple Myeloma

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<p><em>Advances in Hematology</em> publishes original research articles and&nbsp;review articles in all areas of hematology.</p>


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Advances in Hematology publishes original research articles and review articles in all areas of hematology.

Advances in Hematology