Hematology E-Book

Barbara J. Bain

• Herausgeber: John Wiley & Sons
• Kategorie: Fachliteratur
• Sprache: Englisch

Seitenzahl: 232

Veröffentlichungsjahr: 2023

Beschreibung

Hematology Timely morphology resource based on ongoing series of morphology updates published in the American Journal of Hematology since 2008 This is the first book of its kind, written by renowned author Professor Barbara J. Bain, featuring a collection of instructive cases with interesting morphological features initially published in the American Journal of Hematology. This new book aims to bring these interesting and instructive cases to a wider readership. This book features updated cases and a "Test Yourself" section to aid in reader comprehension and information retention. Cases covered in Hematology: 101 Morphology Updates include: * The significance of irregularly contracted cells and hemighosts in sickle cell disease, and striking dyserythropoiesis in sickle cell anemia following an aplastic crisis * Prominent Howell-Jolly bodies when megaloblastic anemia develops in a hyposplenic patient, and unusual aspects of G6PD deficiency * The cause of sudden anemia revealed by the blood film, chorea-acanthocytosis and dysplastic neutrophils in an HIV-positive woman * Neutrophil dysplasia induced by granulocyte colony-stimulating factor, and diagnosis of pyrimidine 5'-nucleotidase deficiency suspected from a blood film Hematology: 101 Morphology Updates is a key resource for consultant hematologists and clinical scientists, trainee hematologists and biomedical scientists. The audience may use this book to solve difficult diagnostic problems or as a source of teaching cases: for both personal learning, including exam revision or solving difficult cases, and as a teaching resource.

Leseprobe

Table of Contents

Cover

Title Page

Copyright Page

Preface

Note to the reader

Acknowledgements

Abbreviations

1 Malaria – one swallow makes a summer

2 The significance of irregularly contracted cells and hemighosts in sickle cell disease

Reference

3 Striking dyserythropoiesis in sickle cell anemia following an aplastic crisis

4 A normal mean cell volume does not exclude a diagnosis of megaloblastic anemia

Reference

5 Prominent Howell–Jolly bodies when megaloblastic anemia develops in a hyposplenic patient

6 A ghostly presence – G6PD deficiency

Reference

7 G6PD deficiency in patients identified as female

8 The cause of sudden anemia revealed by the blood film

9 Choreo‐acanthocytosis

Reference

10 Lead poisoning

References

11 Dysplastic neutrophils in an HIV‐positive woman

Reference

12 Help with HELLP

13 Neutrophil dysplasia induced by granulocyte colony‐stimulating factor

References

14 COVID‐19 and acute kidney injury

References

15 Diagnosis of pyrimidine 5′ nucleotidase deficiency suspected from a blood film

16 Bone marrow aspirate in Chédiak–Higashi syndrome

17 Phytosterolemia

Reference

18 Pseudo‐Chédiak–Higashi inclusions together with Auer rods in acute myeloid leukemia

References

19 Botryoid nuclei resulting from cocaine abuse

References

20 Infantile pyknocytosis

References

21 Splenic rupture in cytomegalovirus infection

References

22 A new diagnosis of monoclonal B‐cell lymphocytosis with cytoplasmic inclusions in a patient with COVID‐19

23 Pseudoplatelets and apoptosis in Burkitt lymphoma

References

24 What is a promonocyte?

References

25 Persistent neonatal jaundice resulting from hereditary pyropoikilocytosis

Reference

26 Auer rods or McCrae rods?

References

27 Observation of Auer rods in crushed cells in acute promyelocytic leukemia

Reference

28 Alpha chain inclusions in peripheral blood erythroblasts and erythrocytes

Reference

29 Dyserythropoiesis in visceral leishmaniasis

Reference

30 Compound heterozygosity for hemoglobins S and D

Reference

31 Granular B lymphoblastic leukemia

References

32 Hyposplenism in adult T‐cell leukemia/lymphoma

33 Voxelotor in sickle cell disease

References

34 The importance of a negative image

35 Seeing what isn’t there

36 A young woman with sudden onset of a severe coagulation abnormality

37 Immature

Plasmodium falciparum

gametocytes in bone marrow

Reference

38 Acute myeloid leukemia with myelodysplasia‐related changes showing basophilic differentiation

References

39 Thiamine‐responsive megaloblastic anemia in an Iraqi girl

40 Teardrop poikilocytes in metastatic carcinoma of the breast

41 A blood film that could have averted a splenectomy

References

42 Russell bodies and Mott cells

References

43 Dutcher bodies

Reference

44 Acute myeloid leukemia with inv(16)(p13.1q22)

Reference

45 Dysplastic macropolycytes in myelodysplasia‐related acute myeloid leukemia

46 Diagnosis of cystinosis from a bone marrow aspirate

47 Emperipolesis in a patient receiving romiplostim

References

48 Mechanical hemolysis: a low mean cell volume does not always represent microcytosis

49 Transplant‐associated thrombotic microangiopathy

References

50 Neuroblastoma in the bone marrow

51 Gray platelet syndrome

52 Diagnosis of systemic lupus erythematosus from a bone marrow aspirate

References

53 Diagnosis from a blood film following a dog bite

54 Interpreting a postpartum Kleihauer test

55 Dengue fever in returning travellers

References

56 Auer rod‐like inclusions in multiple myeloma

References

57 Azurophilic granules in myeloma cells

References

58

Plasmodium knowlesi

59 The cytological features of

NPM1

‐mutated acute myeloid leukemia

References

60 Irregularly contracted cells in Wilson disease

61 Pseudo‐Pelger–Huët neutrophil morphology due to sodium valproate toxicity

62 The distinctive cytological features of T‐cell prolymphocytic leukemia

Reference

63 Eosinophil morphology in the reactive eosinophilia of Hodgkin lymphoma

Reference

64 Malaria pigment

References

65 Salmonella colonies in a bone marrow film

66 Severe babesiosis due to

Babesia divergens

acquired in the UK

References

67 Congenital acute megakaryoblastic leukemia

References

68 Basophilic differentiation in transient abnormal myelopoiesis

69 Methylene blue‐induced Heinz body hemolytic anemia in a premature neonate

References

70 Neutrophil vacuolation in acetominophen‐induced acute liver failure

71 Howell–Jolly bodies in acute hemolytic anemia

72 The distinctive micromegakaryocytes of transformed chronic myeloid leukemia

73 Copper deficiency

74 Chronic neutrophilic leukemia

References

75 Neutrophilic leukemoid reaction in multiple myeloma

References

76 Persistent polyclonal B lymphocytosis

77 Non‐hemopoietic cells in the blood and bone marrow

References

78 It’s a black day – metastatic melanoma in the bone marrow

References

79 Dehydrated hereditary stomatocytosis

80 Circulating lymphoma cells in intravascular large B‐cell lymphoma

81 Unusual inclusions in hemoglobin H disease post‐splenectomy

82 An unexpectedly bizarre blood film in hemoglobin H disease

References

83 Acute myeloid leukemia with a severe coagulopathy and t(8;16)(p11;p13)

References

84 Cold autoimmune hemolytic anemia secondary to atypical pneumonia

85 A confusing ‘white cell count’ – circulating micromegakaryocytes in post‐thrombocythemia myelofibrosis

86 Diagnosis of follicular lymphoma from the peripheral blood

87 Transformation of follicular lymphoma

Reference

88 Cytology of systemic mastocytosis

89 Systemic mastocytosis – the importance of looking within bone marrow fragments

90 Schistocytosis is not always microangiopathic hemolytic anemia

91 Hemoglobin C disease

Reference

92 Hemoglobin St Mary’s

Reference

93 Congenital sideroblastic anemia in a female

Reference

94 A puzzling case of methemoglobinemia

95 Hodgkin lymphoma in a bone marrow aspirate

96 Giant proerythroblasts in pure red cell aplasia due to parvovirus B19 infection in a patient with rheumatoid arthritis

97 A myeloid neoplasm with

FIP1L1::PDGFRA

presenting as acute myeloid leukemia

Reference

98 Breast implant‐associated anaplastic large cell lymphoma

99 Large granular lymphocytosis induced by dasatinib

References

100 The distinctive cytology and disease evolution of blastic plasmacytoid dendritic cell neoplasm

References

101 Platelet phagocytosis as a cause of pseudothrombocytopenia

Test yourself

Answers to test cases

Index

End User License Agreement

Guide

Cover Page

Title Page

Copyright Page

Preface

Note to the reader

Acknowledgements

Abbreviations

Table of Contents

Begin Reading

Test yourself

Answers to test cases

Index

Wiley End User License Agreement

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Hematology

101 Morphology Updates

Barbara J. Bain MBBS, FRACP, FRCPath

Professor of Diagnostic Haematology

St Mary’s Hospital Campus of Imperial College London

and

Honorary Consultant Haematologist

St Mary’s Hospital

Imperial College Healthcare NHS Trust

London

This edition first published 2023© 2023 John Wiley & Sons Ltd

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The right of Barbara J. Bain to be identified as the author of the work has been asserted in accordance with law.

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Library of Congress Cataloging‐in‐Publication Data Applied forHardback: 9781394179817

Cover Design: WileyCover Image: © Roger Sutcliffe/Getty Images

Preface

This book is based on an ongoing series of Morphology Updates that have been published monthly in the American Journal of Hematology since 2008. When necessary, cases have been updated and a test yourself section has been added. The aim of the book is to bring the importance of hematological morphology to a wider readership. In selecting cases for inclusion, preference has been given to those where microscopy was crucial for diagnosis and where there is a generalizable message.

Note to the reader

Unless otherwise stated, all photomicrographs have been stained with a May–Grünwald–Giemsa or similar Romanowsky‐type stain. Photography has generally been with a ×100 objective but sometimes ×50 or other magnification.

Acknowledgements

I wish to acknowledge the Editor of the American Journal of Hematology, Carlo Brugnara, whose idea it was to gather Morphology Updates into book form and thus disseminate them more widely.

The major role of the co‐authors of the original reports is gratefully acknowledged. They are listed alphabetically below, in groups according to their affiliation at the time of writing the initial update, and the original article is cited with each Update.

Co‐authors who are or were, at the time of writing the Update, attached to Imperial College London or Imperial College Healthcare NHS Trust (St Mary’s Hospital, Hammersmith Hospital and Charing Cross Hospital)

Saad Abdalla, Syed Ahmed, Jane Apperley, Marc Arca, Maria Atta, Anna Austin, Peter Bain, Vandana Bharadwaj, Eimear Brannigan, Loretta Brown, Victoria Campbell, Aristeidis Chaidos, Subarna Chakravorty, Lynda Chapple, Kan Cheung, Lucy Cook, Nichola Cooper, Christina Crossette‐Thambiah, Josu De La Fuente, Simona Deplano, Nadine Farah, Rashpal Flora, Rodney Foale, Emma Fosbury, Jacob Grinfeld, Kamala Gurung, Sophie Hanina, Amanda Hann, Andrew Hastings, Marc Heller, Leena Karnik, Mark Layton, Thomas Lofaro, Kirstin Lund, Asad Luqmani, Sasha Marks, Philippa May, Dragana Milojkovic, Audrey Morris, Jane Myburgh, Elizabet Nadal‐Melsió, David Nam, Akwasi Osei‐Yeboah, Bella Patel, Jiří Pavlů, Lorry Phelan, Amin Rahemtulla, Edward Renaudon‐Smith, John Riches, Lynn Robertson, Megan Rowley, Gayathriy Sivaguru, Michael Spencer‐Chapman, Sree Sreedhara, Matthew Stubbs, Sarmad Toma, James Uprichard, Lewis Vanhinsbergh, Vanlata Varu and Eva Yebra‐Fernandez.

Co‐authors attached to other UK hospitals and institutions

Bahaa Al‐Bubseree, Beatson West of Scotland Cancer Centre, Glasgow;Magda Al Obaidi, West Middlesex University Hospital, Isleworth, London;Hannah Al‐Yousuf, North Middlesex University Hospital, London;Philip Ancliff, Great Ormond Street Hospital for Children, London;Anna Babb, West Middlesex University Hospital, Isleworth, London;Linda Barton, University Hospitals of Leicester NHS Trust, Leicester;Tanya Bernard, Ashford and St Peter’s Hospitals NHS Foundation Trust, Chertsey;Manju Bhavnani, Royal Albert Edward Infirmary, Wigan;Kieran Burton, Wycombe Hospital, Buckinghamshire Healthcare NHS Trust, High Wycombe;Carolyn Campbell, Oxford Genetics Laboratories, Oxford University Hospitals NHS Trust, Oxford;Wei Yee Chan, University College London Hospitals NHS Foundation Trust, London;Peter Chiodini, Hospital for Tropical Diseases, London;Barnaby Clark, King’s College and King’s College Hospital, London;Nicholas Cross, Wessex Regional Genetics Laboratory, Salisbury District Hospital, Salisbury;Helen Eagleton, Wycombe Hospital, Buckinghamshire Healthcare NHS Trust, High Wycombe;Emilia Escuredo, St Thomas’ Hospital, London;Rachel Farnell, Wycombe Hospital, Buckinghamshire Healthcare NHS Trust, High Wycombe;Nicholas Fordham, St Helier Hospital, Carshalton, London;Niharendu Ghara, North Middlesex University Hospital, London;Kirsteen Harper, Beatson West of Scotland Cancer Centre, Glasgow;David Hopkins, Beatson West of Scotland Cancer Centre, Glasgow;Ann Hunter, University Hospitals of Leicester NHS Trust, Leicester;Vishal Jayakar, Kingston Hospital, Kingston upon Thames, London;Rosie Jones, Borders General Hospital, Melrose;Andrew Keenan, North Devon District Hospital, Barnstaple;Ahmad Khoder, West Middlesex University Hospital, Isleworth, London;May‐Jean King, NHS Blood and Transplant, Bristol;Victoria Kronsten, King’s College Hospital, London;Alison Laing, Beatson West of Scotland Cancer Centre, Glasgow;Mike Leach, Beatson West of Scotland Cancer Centre, Glasgow;Christine Liu, West Middlesex University Hospital, Isleworth, London;John Luckit, North Middlesex University Hospital, London;Caitlin MacDonald, North Devon District Hospital, Barnstaple;Louisa McIlwaine, Beatson West of Scotland Cancer Centre, Glasgow;Francis Matthey, Chelsea and Westminster Hospital, London;Sajir Mohamedbhai, North Middlesex University NHS Trust, London;Veselka Nikolova, Royal Marsden Hospital, Sutton;Simon O'Connor, Royal Marsden Hospital, Sutton;Katrina Parsons, Beatson West of Scotland Cancer Centre, Glasgow;Sophie Portsmore, North Middlesex University Hospital, London;Clare Rees, Frimley Park Hospital, Camberley;Debbie Shawcross, King’s College Hospital, London;Giulia Simini, West Middlesex University Hospital, Isleworth, London;Wenchee Siow, Wycombe Hospital, Buckinghamshire Healthcare NHS Trust, High Wycombe;Dean Smyth, Beatson West of Scotland Cancer Centre, Glasgow;Simon Stern, St Helier Hospital, Carshalton, London;John Swansbury, The Royal Marsden Hospital, Sutton;Sabita Uthaya, Chelsea and Westminster Hospital, London;Godhev Vijay, King’s College Hospital, London;Barbara Wild, King’s College Hospital, London;Ke Xu, University College London Hospitals NHS Foundation Trust, London;and Anne Yardumian, North Middlesex University Hospital, London.

Co‐authors from Australia

Alan Mills, Bendigo, Victoria (now deceased);Robyn Wells, Princess Alexandra Hospital, Woolloongabba, Queensland;Bronwyn Williams, Royal Brisbane and Women’s Hospital, Herston, Queensland;and Hui Sien Tay, Bendigo, Victoria.

Co‐author from France

Jean‐Baptiste Rieu, Cancer University Institute of Toulouse Oncopole, Toulouse.

Co‐author from India

Biswadip Hazarika, Batra Hospital and Medical Research Centre, New Delhi.

Co‐authors from Iraq

Abbas Hashim Abdulsalam, Al‐Yarmouk Teaching Hospital, Baghdad;Abdulsalam Hatim, National Center for Hematology, Baghdad;Zead Ibrahim, Al‐Yarmouk Teaching Hospital, Baghdad;Mohammed Khamis, Al‐Khadimiya Teaching Hospital, Baghdad;and Nafila Sabeeh, Al‐Yarmouk Teaching Hospital, Baghdad.

Co‐authors from Italy

Francesca Barducchi, Anatomical Pathology, ASL2 Liguria;Enrico Cappelli, Clinical Pathology, ASL2 Liguria;Daniele Delli Carri, Azienda Ospedaliera G. Rummo, Benevento;Maurizio Fumi, Azienda Ospedaliera G. Rummo, Benevento;Brisejda Koroveshi, Clinical Pathology, ASL2 Liguria;Lorella Lanza, Anatomical Pathology, ASL2 Liguria;Flavia Lillo, Clinical Pathology, ASL2 Liguria;Ylenia Pancione, Azienda Ospedaliera G. Rummo, Benevento;Vincenzo Rocco, Azienda Ospedaliera G. Rummo, Benevento;Silvia Sale, Azienda Ospedaliera G. Rummo, Benevento;and Ezio Venturino, Anatomical Pathology, ASL2 Liguria.

Co‐author from Kuwait

Hassan A. Al‐Jafar, Amiri Hospital, Kuwait City.

Co‐authors from Portugal

Rui Barreira, Instituto Português de Oncologia de Lisboa Francisco Gentil (IPOLFG), Lisbon;José Cortez, Instituto Português de Oncologia de Lisboa Francisco Gentil (IPOLFG), Lisbon;Filipa Fernandes, Instituto Português de Oncologia de Lisboa Francisco Gentil (IPOLFG), Lisbon;Rita Ramalho, Instituto Português de Oncologia de Lisboa Francisco Gentil (IPOLFG), Lisbon;and Margarida Silveira, Instituto Português de Oncologia de Lisboa Francisco Gentil (IPOLFG), Lisbon.

Co‐authors from Spain

Beatriz Bua, Hospital Clínico Universitario de Salamanca, Salamanca;Félix Cadenas, Hospital Clínico Universitario de Salamanca, Salamanca;and María Campelo, Hospital Clínico Universitario de Salamanca, Salamanca.

Abbreviations

ADAMTS13

a disintegrin and metalloprotease domain with thrombospondin type 1 motif, member 13

AIDS

acquired immune deficiency syndrome

ALL

acute lymphoblastic leukemia

AML

acute myeloid leukemia

ANAE

α naphthyl acetate esterase

APL

acute promyelocytic leukemia

APTT

activated partial thromboplastin time

ATLL

adult T‐cell leukemia/lymphoma

ATP

adenosine triphosphate

ATRA

all‐

trans

‐retinoic acid

BIA‐ALCL

breast implant‐associated anaplastic large cell lymphoma

BPDCN

blastic plasmacytoid dendritic cell neoplasm

B‐PLL

B‐cell prolymphocytic leukemia

CD

cluster of differentiation

CLL

chronic lymphocytic leukemia

CMV

cytomegalovirus

CNL

chronic neutrophilic leukemia

COVID‐19

corona virus disease 2019

CVAD

cyclophosphamide, vincristine, doxorubicin (Adriamycin) and dexamethasone

2,3‐DPG

2,3‐diphosphoglycerate

DIC

disseminated intravascular coagulation

DNA

deoxyribonucleic acid

EBER

Epstein–Barr virus‐encoded small RNA

EBV

Epstein–Barr virus

EDTA

ethylenediaminetetra‐acetic acid

EMA

eosin‐5′‐maleimide

FAB

French–American–British (classifications of hematological neoplasms)

FEU

fibrinogen equivalent units

FISH

fluorescence

in situ

hybridization

G6PD

glucose‐6‐phosphate dehydrogenase

G‐CSF

granulocyte colony‐stimulating factor

GVHD

graft‐versus‐host disease

H&E

hematoxylin and eosin

Hb

hemoglobin concentration

Hct

hematocrit

HELLP

hemolysis, elevated liver enzymes and low platelet count (syndrome)

HIV

human immunodeficiency virus

HLA

histocompatibility locus antigen

HPLC

high performance liquid chromatography

HTLV‐1

human T‐cell lymphotropic virus 1

Ig

immunoglobulin

IL

interleukin

IRF4

interferon regulatory factor 4

ITD

internal tandem duplication

ITP

immune thrombocytopenic purpura/autoimmune thrombocytopenic purpura

LDH

lactate dehydrogenase

LE

lupus erythematosus

MBCL

monoclonal B‐cell lymphocytosis

MCF

mean cell fluorescence

MCH

mean cell hemoglobin

MCHC

mean cell hemoglobin concentration

MCV

mean cell volume

MDS

myelodysplastic syndrome/s

mRNA

messenger ribonucleic acid

MUM1

multiple myeloma oncogene 1

NADPH

nicotinamide adenine dinucleotide phosphate

NK

natural killer (cell)

NRBC

nucleated red blood cell/s

P5′N

pyrimidine 5′ nucleotidase

PAS

periodic acid–Schiff (reaction)

PCH

paroxysmal cold hemoglobinuria

PCR

polymerase chain reaction

PLT

platelet

PT

prothrombin time

RBC

red blood cell count

RDW

red cell distribution width

RT‐PCR

reverse transcriptase polymerase chain reaction

SARS‐CoV‐2

severe acute respiratory distress syndrome corona virus 2

SLE

systemic lupus erythematosus

Sp

spectrin

SUV

standardized uptake value

TA‐TMA

transplant‐associated thrombotic microangiopathy

TNCC

total nucleated cell count

T‐PLL

T‐cell prolymphocytic leukemia

TTP

thrombotic thrombocytopenic purpura

VEGF

vascular endothelial growth factor

WBC

white blood cell count

WHO

World Health Organization

1Malaria – one swallow makes a summer

A Nigerian woman who was 37 weeks’ pregnant, recently arrived in the UK, presented to a general practitioner with tiredness and dyspnea. Her automated full blood count showed a ‘white cell count’ of 112 × 109/l, Hb 55 g/l, MCV 101 fl and platelet count 471 × 109/l. Examination of a blood film showed that the elevated ‘white cell count’ was due to large numbers of nucleated red blood cells and the true white cell count was 10.7 × 109/l. In addition, the film showed features of sickle cell disease (left image) and high performance liquid chromatography showed hemoglobin S as the major hemoglobin with no hemoglobin A being present. The lack of microcytosis indicated that the diagnosis was sickle cell anemia (SS) rather than compound heterozygosity for hemoglobin S and β0 thalassemia. The patient, when questioned, stated that she had sickle cell trait and denied any knowledge of a diagnosis of sickle cell anemia.

Since the Hb was somewhat lower than expected (although compatible with sickle cell anemia in late pregnancy) the blood film was further examined to try to identify any other factors contributing to the anemia. There was marked polychromasia. There were no hypersegmented neutrophils and the MCV was considered compatible with the reticulocytosis. Unexpectedly, a Plasmodium falciparum ring form was detected (right). A careful search of the film disclosed a total of four parasites. A Giemsa stain showed Maurer clefts and immunological tests for an antigen specific to P. falciparum confirmed the diagnosis. Further questioning of the patient, who was afebrile, disclosed that 3 weeks earlier she had suspected that she had malaria and had taken a single tablet of Fansidar (pyrimethamine plus sulfadoxine) plus a paracetamol tablet. Further appropriate treatment for falciparum malaria was given.

It is stated that ‘one swallow does not a summer make’ but the detection of a single parasite does permit a diagnosis of malaria.

Original publication: Bain BJ (2012) Malaria – one swallow makes a summer. Am J Hematol, 87, 190.

2The significance of irregularly contracted cells and hemighosts in sickle cell disease

The presence of considerable numbers of irregularly contracted cells and hemighosts in sickle cell disease may serve as a warning of severe sickle crisis with significant hypoxia. These two cases demonstrate this association.

The first patient was a 52‐year‐old woman with sickle cell/β0 thalassemia who presented with generalized bone pain. She was given intravenous fluids, antibiotics and analgesics but, despite treatment, developed respiratory failure with a PO2 of 7.0 kPa. Her blood count showed WBC 10.0 × 109/l, Hb 72 g/l, MCV 63.9 fl and platelets 257 × 109/l. In addition to the usual features of sickle cell disease, her blood film showed hemighost cells in which the hemoglobin was retracted to one side of the erythrocyte (top right image). This phenomenon was also observed in cells showing evidence of hemoglobin polymerization – cells with pointed ends and sometimes a gentle curve (top left). She declined continuous positive airway pressure and was commenced on high flow oxygen by nasal cannula, to which she responded well.

The second patient was a young man with sickle cell anemia. He suffered a cardiac arrest and became severely hypoxic. His blood count then showed WBC 26.5 × 109/l, Hb 101 g/l, MCV 88.7 fl and platelet count 110 × 109/. His blood film showed numerous hemighosts with hemoglobin retracted to one side of the cell or to both ends of an elongated cell (bottom images). In addition, there were irregularly contracted cells, some of which were somewhat angular, suggesting that polymerization was occurring (bottom left).

Irregularly contracted cells and hemighosts can be a warning sign of severe hypoxia and worsening crisis in patients with sickle cell disease. This blood film observation reflects the increased percentage of dense cells demonstrable on density gradient analysis in patients with sickle cell crisis.1

Original publication: Siow W, Matthey F and Bain BJ (2017) The significance of irregularly contracted cells and hemighosts in sickle cell disease. Am J Hematol, 92, 966–967.

Reference

1

Fabry ME and Kaul DK (1991) Sickle cell vaso‐occlusion.

Hematol Oncol Clin North Am

,

5

, 375–398.

3Striking dyserythropoiesis in sickle cell anemia following an aplastic crisis

A 7‐year‐old boy with clinically mild sickle cell anemia was transferred to our pediatric intensive care unit from his local hospital. He had presented with fatigue, had developed respiratory distress and had been found to have metabolic acidosis, markedly elevated troponin and severe anemia. His Hb was 20 g/l, with an inadequate reticulocyte response (reticulocyte count 32 × 109/l). He had been transfused 20 ml/kg of O RhD‐negative packed red cells and had been commenced on broad‐spectrum antimicrobials prior to transfer. On arrival he was hemodynamically stable and self‐ventilating.

A chest X‐ray demonstrated right‐sided consolidation. An echocardiogram showed mild mitral regurgitation and tricuspid regurgitation with a dilated left ventricle and mildly reduced ventricular function. Infection screening including urine legionella and pneumococcal antigen, blood cultures, urine cultures and viral respiratory screen were all negative. The patient was intubated and ventilated shortly after arrival in the pediatric intensive care unit. He improved clinically over the next few days, following further red cell transfusion and escalation of antibiotics, and was extubated. Serum troponin gradually normalized. As he recovered, there was an outpouring of nucleated red blood cells into the peripheral blood, with striking dyserythropoiesis. Erythroblasts showed nuclear lobulation, basophilic stippling, detached nuclear fragments, occasional binucleated forms and occasional mitotic figures (images).

Parvovirus B19 serology, IgG and IgM, was positive, confirming recent parvovirus infection. Thus the suspected diagnosis of parvovirus‐induced severe aplastic crisis was confirmed, this being complicated by pneumonia and by cardiac ischemia secondary to severe anemia.

Dyserythropoiesis has many causes, including hemolytic anemia. When erythropoiesis is very active – ‘stress erythropoiesis’ – dyserythropoiesis can be striking, as in this patient. It is important to be aware of the many potential causes of dyserythropoiesis in order to avoid misdiagnosis.

Original publication: Austin A, Lund K and Bain BJ (2019) Striking dyserythropoiesis in sickle cell anemia following an aplastic crisis. Am J Hematol, 94, 378.

4A normal mean cell volume does not exclude a diagnosis of megaloblastic anemia

A 61‐year‐old male was referred to the emergency department by his general practitioner because of pancytopenia. This had been noted several months previously but the patient had refused detailed investigation or treatment. He was noted to have hyperpigmentation of his hands with palmer and plantar freckling. His blood count showed WBC 1.9 × 109/l, neutrophils 0.8 × 109/l, RBC 1.69 × 1012/l, Hb 42 g/l, MCV 81.5 fl, MCH 28 pg, MCHC 343 g/l, red cell distribution width (RDW) 35.5% (normal range 10–16), platelets 47 × 109/l and reticulocytes 44.5 × 109/l. His blood film (all images) showed marked anisocytosis and poikilocytosis, red cell fragments, macrocytes, oval macrocytes, teardrop poikilocytes and Howell–Jolly bodies (bottom right). Biochemical tests showed lactate dehydrogenase 5594 iu/l, creatinine 84 μmol/l, bilirubin 59 μmol/l and ferritin 257 μg/l. Serum haptoglobin was 0 g/l (0.52–2.24). A coagulation screen was normal.