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Hematology Reports, Volume 14, Issue 4

2022 December - 13 articles

Cover Story: Medich syndrome is a rare macrothrombocytopenia with unusual scroll-like platelet inclusions in cross-sections; they are tubular or cone-shaped longitudinally and may deform a cell’s discoid shape. At the periphery of the inclusions, numerous layers of membranes and aggregates of glycogen are also present.  First described by Dr. James G. White in 2004, the condition also includes a deficiency of alpha granules and occasional giant alpha granules, as seen in chromosome 11q deletion syndrome. Of five cases reported to date, three presented with thrombocytopenia at birth, another at two weeks, and one at a year of age. All have been transfusion-dependent, and the diagnosis of Medich syndrome took years to establish. There is no specific genetic mutation identified yet for the syndrome. View this paper
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Articles (13)

  • Article
  • Open Access
7 Citations
2,915 Views
12 Pages

12 December 2022

Background: Immunocompromised patients, including those with hematological malignancies, are at a high risk of developing severe coronavirus disease 2019 (COVID-19) complications. Currently, there is a limited number of systematic reviews into the ef...

  • Case Report
  • Open Access
2,345 Views
4 Pages

5 December 2022

Patients with protein S (PS) deficiency possibly have a higher risk of developing severe COVID-19 disease. Therefore, vaccination against SARS-CoV-2 infections is recommended for PS-deficient patients. However, there are limited data regarding the sa...

  • Review
  • Open Access
9 Citations
4,815 Views
15 Pages

1 December 2022

Adenoviral-based vaccines such as ChadoX1 CoV-19 (AstraZeneca) and Ad26.COV2.S (J&J) were developed to prevent infection and reduce hospitalization or death in Coronavirus Disease 2019 (COVID-19) patients. Although these vaccines passed safety an...

  • Case Report
  • Open Access
2 Citations
3,883 Views
9 Pages

Medich Giant Platelet Syndrome: An Evolving Qualitative and Quantitative Platelet Disorder

  • Gita Massey,
  • Laura Tyrrell,
  • Yaser Diab and
  • William T. Gunning

1 December 2022

Qualitative platelet disorders remain rare and varied. We describe here 2 additional patients with giant platelets, thrombocytopenia, deficiency in alpha granules and the presence of membranous inclusions within the cytoplasm. Collectively known as M...

  • Article
  • Open Access
1 Citations
2,625 Views
7 Pages

Cutaquig® Is Well Tolerated in Immunodeficient Patients Who Did Not Tolerate Other Subcutaneous Immunoglobulin Products

  • Sydney Brownlee,
  • Crystal Allen,
  • Mohammed F. Kana’an,
  • D. William Cameron and
  • Juthaporn Cowan

17 November 2022

Objective: Subcutaneous immunoglobulin (SCIG) treatment is generally tolerable, but some patients may experience adverse events to one or more SCIG products. We investigated whether 16.5% Cutaquig® treatment offered a tolerable and safe alternati...

  • Case Report
  • Open Access
2,591 Views
7 Pages

An Isolated Mesenteric Presentation of a Nodal Peripheral T Cell Lymphoma with T Follicular Helper Cell Phenotype

  • Anna Keogh,
  • Fiona Lynott,
  • Antonios Papanicolau-Sengos,
  • Mutaz Mohammed Nur,
  • Aisling Spillane,
  • Fiona Quinn,
  • Ezzat ElHassadi,
  • Elaine S. Jaffe and
  • Richard Flavin

15 November 2022

Nodal peripheral T cell lymphoma (PTCL) with T follicular helper (TFH) cell phenotype is a provisional entity added to the 2016 revised WHO classification of haematological malignancies. These lymphomas have an aggressive clinical course and respond...

  • Case Report
  • Open Access
1 Citations
4,425 Views
13 Pages

Unusual Presentation of Hodgkin’s Lymphoma in Pregnancy: A Case Report and Systematic Review of Literature

  • Joseph Delzotto,
  • Tahira. S. Naqvi,
  • Nnennaya. U. Opara,
  • Anthony Delzotto and
  • Andrew Morgan

3 November 2022

Diseases occurring during pregnancy create a dilemma of managing the patient without causing harm to the unborn child. Three percent of the peak incidence of Hodgkin lymphoma (HL) is congruent with the reproductive period, particularly with pregnancy...

  • Case Report
  • Open Access
4 Citations
8,350 Views
12 Pages

Acute Promyelocytic Leukemia in a Woman with Thalassemia Intermedia: Case Report and Review of Literature on Hematological Malignancies in β-Thalassemia Patients

  • Claudio Pellegrino,
  • Giulia Dragonetti,
  • Patrizia Chiusolo,
  • Monica Rossi,
  • Nicoletta Orlando and
  • Luciana Teofili

21 October 2022

Patients affected by transfusion-dependent β-thalassemia are prone to developing several clinical complications, mostly related to the iron overload. We report the case of a patient affected by transfusion-dependent β-thalassemia (TDT) deve...

  • Case Report
  • Open Access
2,179 Views
5 Pages

Immense Tumor of Maxillary Sinus with Exophthalmos—A Rare Underlying Cause

  • Olga Lesniewska-Skowerska,
  • Joanna Symela-Kaspera,
  • Lucyna Klimczak-Gołąb,
  • Wojciech Smolka and
  • Jaroslaw Markowski

10 October 2022

Sinus tumors are arduous to diagnose due to often prolonging asymptomatic course until the infiltration of the adjacent structures occurs. Therefore, patients are diagnosed with advanced-stage disease, which negatively affects the treatment outcomes....

  • Case Report
  • Open Access
1 Citations
3,713 Views
5 Pages

Case Report: α-Spectrin Mutation Associated with αLELY Polymorphism Responsible for Hereditary Pyropoikilocytosis

  • María Sánchez Villalobos,
  • Eduardo Salido Fiérrez,
  • Jorge Martínez Nieto,
  • Mª Carmen García Garay,
  • Asunción Beltrán Videla,
  • Ana Belen Pérez Oliva,
  • Miguel Blanquer Blanquer and
  • José María Moraleda Jiménez

8 October 2022

Hereditary pyropoikilocytosis (HPP) is characterised by severe hemolytic anemia due to membrane instability. We report the case of a 13-day-old boy with neonatal jaundice and severe hemolytic anemia. A peripheral smear examination showed severe aniso...

  • Case Report
  • Open Access
3 Citations
5,728 Views
6 Pages

2 October 2022

Classic dyskeratosis congenita is a hereditary disease where the majority of patients present with bone marrow failure and mucocutaneous changes: mainly skin pigmentation, nail dystrophy, oral premalignant leukoplakia, in addition to increased risk f...

  • Case Report
  • Open Access
1 Citations
3,043 Views
4 Pages

Medication-Induced Factor V Inhibition in the Setting of Refractory Coagulopathy

  • Brandon Travis Wiggins,
  • Daniel Ramirez,
  • Daniel Taylor,
  • William Reichardt,
  • Alyssa Kipke and
  • Mark Minaudo

21 September 2022

Liver cirrhosis is commonly associated with coagulopathies, typically demonstrated by elevated prothrombin time, international normalized ratio, and partial thromboplastin time. In the setting of bleeding related to coagulopathies, oftentimes physici...

  • Case Report
  • Open Access
1 Citations
2,840 Views
4 Pages

21 September 2022

Myeloproliferative neoplasms (MPN), which include primary myelofibrosis (PMF) and essential thrombocytopenia (ET), are characterized by the clonal proliferation of mature blood cells as a result of the overactivation of the JAK/STAT pathway. Extramed...

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Hematol. Rep. - ISSN 2038-8330