Hematology Reports

5 pages, 364 KiB

Open AccessArticle

The Use of Mean Platelet Volume for Distinguishing the Causes of Thrombocytopenia in Adult Patients

by Lalita Norrasethada, Wichan Khumpoo, Ekarat Rattarittamrong, Thanawat Rattanathammethee, Chatree Chai-Adisaksopha and Adisak Tantiworawit

Hematol. Rep. 2019, 11(1), 7732; https://doi.org/10.4081/hr.2019.7732 - 29 Mar 2019

Cited by 15 | Viewed by 915

Abstract

Establishment and analysis of mean platelet volume (MPV) may be helpful in the discrimination between underproduction or over-destruction of platelets as the causes of thrombocytopenia. The primary objective is to find the cut-off point of MPV for distinguishing causes of thrombocytopenia. The secondary [...] Read more.

Establishment and analysis of mean platelet volume (MPV) may be helpful in the discrimination between underproduction or over-destruction of platelets as the causes of thrombocytopenia. The primary objective is to find the cut-off point of MPV for distinguishing causes of thrombocytopenia. The secondary objective is to validate the cut-off value of the MPV by using bone marrow examination. Thrombocytopenic patients were enrolled in a training set and a receiving operating characteristics (ROC) curve was plotted to obtain the cut-off value of MPV. A validation set of patients was recruited to validate the cut-off value. The training set included 240 patients. Half with with underproductive (n = 120) and half with over-destructive thrombocytopenia (n = 120). The best cut-off value of MPV was 8.8 fL. The validation set included 119 patients in total, again in 2 groups, those with underproductive (n = 84) and those with overdestructive thrombocytopenia (n = 35). The sensitivity, specificity, PPV and NPV when MPV ≥ 8.8 fL indicating over-destructive thrombocytopenia were 77%, 89%, 89% and 77%, respectively. MPV is useful for differentiating the cause of thrombocytopenia. The value of MPV ≥ 8.8 fL has acceptable sensitivity and specificity for diagnosis of over-destructive thrombocytopenia. Full article

3 pages, 316 KiB

Open AccessCase Report

Relapsing Thrombotic Thrombocytopenic Purpura with Low ADAMTS13 Antigen Levels: An Indication for Splenectomy?

by Anna Maria Lombardi, Irene Di Pasquale, Maria Antonietta Businaro, Irene Cortella, Silvia Ferrari, Fabrizio Fabris and Fabrizio Vianello

Hematol. Rep. 2019, 11(1), 7904; https://doi.org/10.4081/hr.2019.7904 - 19 Mar 2019

Cited by 2 | Viewed by 433

Abstract

With more recent modalities of immunosuppression, splenectomy is now rarely considered in refractory/relapsed thrombotic thrombocytopenic purpura (TTP). However, the surgical approach had shown convincing evidences of high efficacy in the pre-rituximab era and therefore may still represent a lifesaving option in selected challenging [...] Read more.

With more recent modalities of immunosuppression, splenectomy is now rarely considered in refractory/relapsed thrombotic thrombocytopenic purpura (TTP). However, the surgical approach had shown convincing evidences of high efficacy in the pre-rituximab era and therefore may still represent a lifesaving option in selected challenging cases. To define the characteristics of subjects who may benefit from splenectomy may ease clinical decision making. In this paper we describe the clinical and laboratory data of 2 multiple relapsing TTP cases who successfully underwent splenectomy in the pre-rituximab era. Whereas high anti-ADAMTS13 antibody titre and low ADAMTS13 activity never correlated with remission and relapse, a drop in the ADAMTS13 antigen level was always associated with the acute phase, whereas levels consistently returned to normal following splenectomy, heralding long term remission. Splenectomy may therefore be considered in refractory TTP cases associated with increased ADAMTS13 antigen clearance, irrespective of persistence of inhibitory antibodies. Full article

4 pages, 374 KiB

Open AccessCase Report

MPL Y252H and MPL F126fs Mutations in Essential Thrombocythemia: Case Series and Review of Literature

by Ahmed G. Elsayed, Aeesha Ranavaya and Muhammad Omer Jamil

Hematol. Rep. 2019, 11(1), 7868; https://doi.org/10.4081/hr.2019.7868 - 12 Mar 2019

Cited by 6 | Viewed by 601

Abstract

Essential thrombocythemia (ET) is a clonal bone marrow disease, characterized by increased production of platelets along with other clinical and bone marrow findings. Most patients with ET will have a somatic mutation in one of the known gene locations of JAK2, CALR , [...] Read more.

Essential thrombocythemia (ET) is a clonal bone marrow disease, characterized by increased production of platelets along with other clinical and bone marrow findings. Most patients with ET will have a somatic mutation in one of the known gene locations of JAK2, CALR , or MPL that can upregulate the JAK-STAT pathway. MPL mutation is present in 5% of cases with the most common mutations being W515L and W515K. In this report we describe 2 cases of patients with clinical and laboratory picture of ET. One patient carried MPLY252H mutation which is previously unreported in the adult population but has been shown to be a gain-of-function mutation. The other patient carried MPL F126fs mutation which is not known to be of clinical importance and has not been previously reported. Full article

4 pages, 364 KiB

Open AccessArticle

Studying Anemia of Chronic Disease and Iron Deficiency in Patients with Rheumatoid Arthritis by Iron Status and Circulating Hepcidin

by Warqaa Khalaf, Haithem Ahmed Al-Rubaie and Sami Shihab

Hematol. Rep. 2019, 11(1), 7708; https://doi.org/10.4081/hr.2019.7708 - 12 Mar 2019

Cited by 13 | Viewed by 990

Abstract

Anemia of chronic disease (ACD) and iron deficiency anemia (IDA) are the two most important types of anemia in rheumatoid arthritis (RA). Functional iron deficiency in ACD can be attributed to overexpression of the main iron regulatory hormone hepcidin leading to diversion of [...] Read more.

Anemia of chronic disease (ACD) and iron deficiency anemia (IDA) are the two most important types of anemia in rheumatoid arthritis (RA). Functional iron deficiency in ACD can be attributed to overexpression of the main iron regulatory hormone hepcidin leading to diversion of iron from the circulation into storage sites resulting in iron-restricted erythropoiesis. The aim is to investigate the role of circulating hepcidin and to uncover the frequency of IDA in RA. The study included 51 patients with RA. Complete blood counts, serum iron, total iron binding capacity, ferritin, and hepcidin- 25 were assessed. ACD was found in 37.3% of patients, IDA in 11.8%, and combined (ACD/IDA) in 17.6%. Serum hepcidin was higher in ACD than in control and the other groups (P ≤ 0.001). It was strongly and positively correlated with ferritin (P < 0.001), while hemoglobin, serum iron, and total iron binding capacity were negatively correlated with hepcidin (P = 0.016, 0.022 and <0.001, respectively). High serum hepcidin was significantly associated with ACD in RA. IDA alone or combined with ACD was encountered in about a third of patients. Full article

3 pages, 317 KiB

Open AccessCase Report

A Unique Case of Durable Complete Remission after Salvage with Azacitidine and DLI for High Risk FLT-3 Positive Acute Myeloid Leukemia, Following Relapse 18 Months Post Allogeneic Stem Cell Transplant

by Claire Horgan, Alexandros Kanellopoulos, Shankara Paneesha, Bhuvan Kishore, Richard Lovell and Emmanouil Nikolousis

Hematol. Rep. 2019, 11(1), 7800; https://doi.org/10.4081/hr.2019.7800 - 25 Feb 2019

Cited by 2 | Viewed by 439

Abstract

A unique case of primary refractory FLT3-itd mutated acute myeloid leukemia in an elderly patient, who achieved completed morphological remission, and FLT3-itd negativity, following 9 cycles of azacitadine in combination with escalating doses of donor lymphocyte infusions following relapse 18 months post reduced [...] Read more.

A unique case of primary refractory FLT3-itd mutated acute myeloid leukemia in an elderly patient, who achieved completed morphological remission, and FLT3-itd negativity, following 9 cycles of azacitadine in combination with escalating doses of donor lymphocyte infusions following relapse 18 months post reduced intensity HLAA mismatch Campath conditioning allogeneic stem cell transplant. Full article

2 pages, 284 KiB

Open AccessCase Report

Use of Catridecacog in a Patient with Severe Factor XIII Deficiency Undergoing Surgery

by Gianluca Sottilotta, Francesca Luise, Vincenzo Oriana, Angela Piromalli, Rosa Santacroce and Alessandra Di Lelio

Hematol. Rep. 2019, 11(1), 7912; https://doi.org/10.4081/hr.2019.7912 - 19 Feb 2019

Cited by 2 | Viewed by 460

Abstract

Despite many articles regarding the antihemorrhagic treatment and prophylaxis, there is a lack of experience about how to best conduct major surgical procedures in patients with congenital factor XIII (FXIII) deficiency. Here we report a case of surgery (right inguinal hernia, complicated by [...] Read more.

Despite many articles regarding the antihemorrhagic treatment and prophylaxis, there is a lack of experience about how to best conduct major surgical procedures in patients with congenital factor XIII (FXIII) deficiency. Here we report a case of surgery (right inguinal hernia, complicated by heaviness and pain) performed in a patient with FXIII deficiency, receiving recombinant FXIII prophylaxis (Catridecacog 35 UI/kg every 28 ± 2 days). Our experience shows that Catridecacog can be used safely and effectively not only for continued prophylaxis but also in surgery and adds to the very limited body of evidence currently available on surgery in this bleeding disorder. Full article

3 pages, 312 KiB

Open AccessArticle

Patterns of Prescribing Hydroxyurea for Sickle Cell Disease Patients from a Central Hospital, Saudi Arabia

by Assem A. Elghazaly, Adel A. Aljatham, Abdulaziz M. Khan, Rasha M. Elneil, S. Zubair S. Jafar, Salah A. Elwishy, Omsalma E. Ahmed, Aamer M. Bughnah and Mamoun H. Ibrahim

Hematol. Rep. 2019, 11(1), 7860; https://doi.org/10.4081/hr.2019.7860 - 19 Feb 2019

Cited by 3 | Viewed by 533

Abstract

Sickle cell disease (SCD) is a group of inherited disorders of hemoglobin synthesis. It is prevalent in different parts of the world, including the Kingdom of Saudi Arabia. The disease is associated with multiple acute and chronic life-threatening complications. Hydroxyurea (HU) is an [...] Read more.

Sickle cell disease (SCD) is a group of inherited disorders of hemoglobin synthesis. It is prevalent in different parts of the world, including the Kingdom of Saudi Arabia. The disease is associated with multiple acute and chronic life-threatening complications. Hydroxyurea (HU) is an effective preventive medication; its use has resulted in decreased morbidity and mortality. However, practice variability, including underutilization of HU, has been reported. No local publication has addressed this issue. The aim of this work is to consider the pattern of HU prescription for SCD patients. This is a retrospective study included patients seen in the outpatient clinics in a central hospital. Cases of medications unavailability or patient refusal to take the drug were not included. A total of 152 patients were included, of them 118 were prescribed HU and 34 were not. In 133 (87.5%) patients, the physician’s decision was appropriate. Inappropriate decisions including both under prescription and, to much lesser extent, over utilization had been demonstrated in 19 (12.5%) cases. Impact of raising the healthcare providers’ awareness and improving compliance with the updated SCD management recommendations and guidelines deserve further studying. In our local experience, although the majority of HU prescriptions were appropriate, both under prescription and to a lesser extent, overutilization was demonstrated. Full article

3 pages, 441 KiB

Open AccessCase Report

Silent T-Cell Receptor Cutaneous T-Cell Lymphoma Associated to a Clonal Plasma Cell Proliferation

by Ana Caballero, Silvana Novelli, Anna Mozos, Pilar Garcia Muret, Jorge Sierra and Javier Briones

Hematol. Rep. 2019, 11(1), 7841; https://doi.org/10.4081/hr.2019.7841 - 19 Feb 2019

Cited by 5 | Viewed by 723

Abstract

Within T-cell lymphomas (TCL) there are 2 entities expressing gamma-delta TCR: hepatosplenic gamma-delta T-cell lymphoma (HSGDTL) and the primary cutaneous gamma-delta T-cell lymphoma (PCGDTL). PCGDTL is a rare form of T-cell lymphoma with specific tropism for skin that have a dismal prognosis. Although [...] Read more.

Within T-cell lymphomas (TCL) there are 2 entities expressing gamma-delta TCR: hepatosplenic gamma-delta T-cell lymphoma (HSGDTL) and the primary cutaneous gamma-delta T-cell lymphoma (PCGDTL). PCGDTL is a rare form of T-cell lymphoma with specific tropism for skin that have a dismal prognosis. Although even rarer, there have been reports of TCL with loss of expression of the TCR, which have been termed peripheral TCL TCR-silent type. We report the case of a cutaneous TCR-silent type lymphoma associated to a clonal plasma cell proliferation with an ominous outcome that led to a lot of discussion in its classification. Due to the aggressiveness of the disease and the scant evidence about therapy in this strange entity the outcome was fatal. We report a unique case of a TCR-silent cutaneous TCL with an exceptional histopathology, prolonged clinical evolution and a subsequent plasma cell clonal expansion. Full article

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Hematol. Rep., Volume 11, Issue 1 (February 2019) – 8 articles

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