Reliability of leukostasis grading score to identify patients with high‐risk hyperleukocytosis

Abstract

An adult patient affected by b 0 -thalassemia major underwent allogeneic bone marrow transplant (BMT) from a matched related donor. Forty days after transplant, allogeneic engraftment failure and autologous b 0 -thalassemic bone marrow recovery were documented. Red blood cell transfusions were required until 118 days post-transplant. Thereafter, the haemoglobin (Hb) levels stabilized over 11.8 gr/dl throughout the ongoing 34-month follow-up, abolishing the need for transfusion support. The Hb electrophoresis showed 100% Hb Fetal (HbF). This unexplained case suggests full HbF production may occur in an adult patient with b 0 -thalassemia major.Homozygous b-thalassemia is a severe disorder caused by inheritance of two b-thalassemia alleles. The gene mutations produce absent or insufficient synthesis of b-globin chains leading to excess a-globin chain accumulation and precipitation in early erythroid cells [1]. Concurrent genetic factors (hereditary persistence of fetal hemoglobin(HbF), a-gene mutations, and db-thalassemia determinants) are known to modify the globin chain imbalance [1,2].To date, the only cure for thalassemia major is stem cell transplantation [3]. Here, we report an atypical case of sustained and full HbF production after graft failure in an adult b 0 -thalassemic patient.On September 2005, an 18-year-old patient affected by b 0 -thalassemia major was referred to our center for bone marrow transplant (BMT) from an HLA identical sibling. The thalassemia Major diagnosis was evident by 2 years of age when the blood transfusion therapy was started and regularly administered every 2-3 weeks. After splenectomy, performed when the patient was 6 years old, the transfusion requirement was reduced to one red blood cell unit every 2-3 months. Such behavior was maintained during the following years without any transfusion-free period. The iron-chelation therapy was never administered.At the time of presentation to our Institution, the patient presented typical thalassemic facies and hepatomegaly (over 4 cm below the ribs). The complete blood counts showed: low Hb level (9.7 g/dl), low red blood cell count (3.59 3 10 12 /l), a mean corpuscular volume of 82 fl, a mild increase of reticulocyte count (4.07%), increased white cell count (3.901 3 10 12 /l), abundant circulating nucleated red cells (80% of total nucleated cells), and an elevated platelet count (803 3 10 9 /l). The bone marrow exhibited marked hypercellularity. The Hb profile showed predominantly HbF (73.6%), and the genetic analysis detected a double mutation in the b-globin locus: the homozygous mutation IVS-I-1 of the b-globin gene and the homozygous mutation for T variant at position 2158 upstream of the Gg-globin gene. High-performance liquid chromatography (HPLC) analyses detected the lack of b-chain synthesis (b 0%) and 2.47% of the a/non a imbalance.Chronic hepatitis C was documented by a HCV-RNA test. Liver biopsy showed mild fibrosis (1/6 degree) and marked iron overload ([3/4] histological score). The liver iron concentration was marked ...

The effect of therapeutic leukapheresis on early complications and outcomes in patients with acute leukemia and hyperleukocytosis: a propensity score‐matched study

“…We applied a leukostasis grading score (LGS) to enrolled patients to determine the severity of leukostasis. This LGS was developed by Novotny and colleagues to identify the clinical probability of leukostasis, and Piccirillo and coworkers demonstrated good correlation between the LGS and early death …”

How I treat hematologic emergencies in adults with acute leukemia

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Out with the bad and in with the good; red cell exchange, white cell reduction, and platelet reduction

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