Iraqi Journal of Hematology

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder of the hematopoietic stem
cells, which manifests as bone marrow failure, hemolytic anemia, and thrombosis. PNH affects both
sexes equally and may present at any age, although it is most often diagnosed in young adulthood.
Here, we report a case of a middle‑aged woman, presented with one and a half year history of anemia,
significant fatigue, backache, and persistent high erythrocyte sedimentation rate. The patient has
no organomegaly and did not give a history of thrombotic events previously. She was considered
as a case of megaloblastic anemia at another center and treated accordingly. Her full blood count
showed mild macrocytic anemia with slight polychromasia. The hemolytic anemia workup revealed
slightly raised serum bilirubin (unconjugated) and undetectable haptoglobin with hemosiderin in urine.
Ham’s test was positive, and the diagnosis of PNH was confirmed by flow cytometry. The patient
was treated with short‑course steroids and folic acid, and her general condition has become stable.

Abstract:
BACKGROUND: Sickle cell anemia is the most common inheritable hemoglobin disorder in the
world with very high prevalence, morbidity, and mortality in Sub‑Saharan Africa. Acute chest
syndrome (ACS) is one of the most common causes of mortality among individuals with the
condition. The management of this condition involves watchful waiting which may be deleterious in
resource‑constrained settings. The use of exchange blood transfusion (EBT) has been reported to
be beneficial. The use of this great intervention is even further hampered in most of the developing
world. The aim of this study was to review the outcome data of children managed with an algorithm
adopted in 2015 at a University Teaching Hospital in a resource‑constrained setting.
MATERIALS AND METHODS: This was a retrospective study from January 2015 to December
2017 at a University Teaching Hospital. Ethical approval was obtained from the Hospital’s Health,
Research, and Ethics committee.
RESULTS: A total of 324 children with sickle cell disease were admitted in the period under review
with a male‑to‑female ratio of 1.5:1. Thirty‑three patients were suspected of having ACS, but only
12 were confirmed using the algorithm. The mean age at presentation was 8 years with over 85%
of the patients having a triad of fever, cough, and chest pain. All the patients had EBT within 48 h
of admission and mortality rate recorded was 16.7%.
CONCLUSION: The use of the algorithm as well as the preemptive EBT improved the outcome of
the children accessing care at our facility.

BACKGROUND: Acute myeloblastic leukemia is the most common acute leukemia in adults,
with both diagnostic and therapeutic challenges. It has a poor treatment outcome both locally and
internationally. The aim of this study was to investigate some epidemiological aspects and treatment
outcome of AML patients treated in single center.
PATIENTS AND METHODS: A retrospective observational study extended from February 2013
to February 2018 and included 98 patients who referred to Hiwa Hematology Oncology Hospital
in Sulaymaniyah city. All patients had met the diagnostic criteria by flow cytometry for de novo acute
myeloid leukemia (AML). Electronic records of all patients were reviewed carefully.
RESULTS: Ninety‑eight patients (46 females and 52 males) with a mean age of 42 years were included
the study, with new diagnosis of AML, most of the patients were below the age of 60 years (81%),
while only 19% were 60 years of age or older. The most frequent subtype is AML M3 (n = 25; 25.5%)
of patients, followed by M1, M2 (n = 16; 16.3%) each, and M5 (n = 15; 15.3%) with M4, M0, M6,
M7, and acute leukemia of ambiguous lineage comprising the remainder, respectively. The clinical
features at the diagnosis of the 98 patients included pallor in 91 patients (92.9%), easy fatigability
in 82 patients (83.6%), while bleeding tendency was present in 46 patients (46.9%) in the form of
ecchymosis, petechial hemorrhage, epistaxis, or abnormal vaginal bleeding. Fever was present in
85 patients (86.7%), while pain in the form of headache, generalized body ache, or bone pain was
initially manifested in 29 patients (29.6%). The survival of our patients at 1 year is (88%) M3 patients,
while it was (58%) for non‑M3 patient.
CONCLUSIONS: Clinical and epidemiological characteristics were different in some aspect, while
comparable in other when compared to published studies. treatment outcome and survival data
were comparable to international data.

Abstract:
Most of the patients with parvovirus B19 infection manifest with anemia and erythroid hypoplasia in
bone marrow when the immunity of host is not competent enough to kill the virus like in posttransplant
patients on immunosuppressive drugs and immunodeficiency syndromes. Herein, we present a case
of parvovirus infection in an immunocompetent patient presenting with anemia, showing erythroid
hyperplasia with hemophagocytosis in bone marrow, and also discuss the pathogenesis with review
of literature in such a rare presentation.

BACKGROUND: Anthracyclines are antibiotic antineoplastic drugs used in the treatment of various
hematological and solid malignancies. They are well‑known for their cardiotoxic side effects which are
related to their cumulative dosages. Many investigations are used to detect such cardiotoxicity, the
gold standard of which is the endomyocardial biopsy that has a limited use because it is an invasive
procedure. The aims of this study were to explore the cardiotoxicity induced by anthracyclines in
patients with hematological malignancies by using electrocardiography (ECG) and echocardiography
and to determine relationship between the detected changes in ECG and echocardiography, with
some demographic and clinical variables.
PATIENTS AND METHODS: A cross‑sectional study of fifty patients was recruited at Al‑Imamein
Al‑Kadhemein Medical City from March 2014 to January 2015. ECG and echocardiography were done
to the patients to study several parameters such as ejection fraction (EF), diastolic dysfunction, QRS
voltage, QRS duration, and corrected QT (QTc) both at baseline and reassessment. Reassessment
was done after completing induction for leukemia patients and at mid‑term reassessment for
lymphoma patients.
RESULTS: ECG showed reduction in QRS voltage, increase in QRS duration, and increase in
QTc, all of which showed statistical significance and may reflect the effect on the depolarization
and repolarization on the myocardium. The echo study showed the development of systolic left
ventricular (LV) dysfunction (EF <55%) in 12% of patients and a statistically significant reduction
in the mean of EF of the study group. It also showed statistically significant development of new
diastolic dysfunction. Statistically significant association between reduction of QRS voltage (>30%
from baseline) with the development of LV dysfunction was also found.
CONCLUSIONS: Significant changes in QRS voltage, QRS duration, QTc, as well as the occurrence
of LV systolic dysfunction and diastolic dysfunction were noted. The mean cumulative dose at which
LV systolic dysfunction occurred was about 250 mg/m2.

BACKGROUND: Polycythemia vera (PV) is a chronic myeloproliferative stem cell disease usually
combined with JAK2 mutation. Active JAK2 mutation directly promote platelets and granulocytes
activation and indirectly initiate endothelial activation. Such interactions can provoke endothelial
injury with subsequent release of procoagulant factors. The aim of this study was to understand
of this study is to understand the pathophysiology of increased thrombosis in PV in terms of the
effect of JAK2V617F gene mutation in relation to the intensity of the neutrophil CD11b and platelet
CD62P.
SUBJECTS AND METHODS: A group of 53 patients was examined comprised 30 PV patients
and 23 with secondary polycythemia. In parallel, 30 aged‑ and sex‑matched, nonsmokers healthy
volunteers served as a control group. Hemoglobin level, packed cell volume, white and red blood
cells count, mean corpuscular volume, and platelet count were estimated for all the 83 participants.
Flow cytometry for the detection of neutrophil CD11b and platelet CD62P were done for all the
participants; whereas quantitative polymerase chain reaction technique for the assessment of JAK2
mutation rate was done for the 30 PV patients only.
RESULTS: We were able to detect significantly higher neutrophil CD11b and platelet CD62P
expressions in PV patients. Within PV, JAK2 mutation rate was significantly higher in those with a
history of thrombosis. A positive relationship was demonstrated between the JAK2 mutation rate
and each of CD11b and CD62P.
CONCLUSION: There is an overexpression of neutrophil CD11b and platelets CD62P in patients
with PV which can be considered as a marker of procoagulant activity in blood cells. Active JAK2
mutation directly promote platelets and granulocytes activation and indirectly initiate endothelial
activation and in turn endothelium into a proadhesive and procoagulant surface.

Dear Editor,
We read with great interest the article “Clinicohematological
profile of patients with peripheral blood cytopenias in
clinical practice” by Thakur et al.[1] The most common
cause of cytopenias in their study was infections followed
by megaloblastic anemia reflecting the high prevalence
of infections and nutritional deficiency in the developing
countries. Megaloblastic anemia is the most common
cause of pancytopenia in their study as well as from other
studies from the region.[1,2] Another common nutritional
cause of cytopenias is dimorphic anemia.[2] Raphael et al.
reported 8.75% of cases of dimorphic anemia presenting
with pancytopenia and Garg et al. reported 9.55% of cases
presenting with pancytopenia.[2,3] Dimorphic anemia
presenting with bicytopenia was as high as 50.5% in the
series by Garg et al.[3]
Dimorphic anemia, morphologically characterized by
two cell populations, strongly suggests a dual deficiency
of iron and Vitamin B12/folate.[3] Another important
cause of dimorphic anemia is the unmasking of an iron
deficiency following the treatment of megaloblastic
anemia. Although the deficiency of Vitamin B12/folate
is responsible for cytopenias, dimorphic anemia should
be recognized distinct from that of megaloblastic anemia.
The response may not be effective if only Vitamin
B12/folate deficiency is treated.[3] Nutritional anemia,
which is an important cause of dimorphic anemia, is a
significant problem in the Indian subcontinent. However,
dimorphic anemia, which contributes to a significant
chunk of cytopenias, is an undermined entity.

Abstract:
Peutz–Jeghers syndrome (PJS) is a rare disorder, which is inherited as autosomal dominant. It
is characterized by mucocutaneous melanotic spots and multiple hamartomatous polyps with an
increased risk of cancer predispositions. PJS can present with complications such as iron‑deficiency
anemia as a result of bleeding from the gastrointestinal polyps and intestinal obstruction and/or
intussusception if the polyps are of large size. We present a case of PJS with iron‑deficiency
anemia in a 16‑year‑old boy as a result of occult gastrointestinal bleeding from the hamartomatous
gastrointestinal polyps.

Abstract:
BACKGROUND: Thalassemia trait and other low red cell index (LRCI) diseases commonly have
same presentation with microcytic hypochromic anemia. Most of beta thalassemia minor (TM) people
are subclinical and without specific investigation may be undiagnosed or treated as iron‑deficiency
anemia. Thalassemia carriers may be undiagnosed, which in turn leads to severe forms of thalassemia
syndromes with poor premarital counseling in high‑prevalence areas. Many trials tried to find simple
diagnostic tools to differentiate between thalassemia traits and other microcytic anemia depending
on blood discriminative indices that can be found in limited resource places and routine clinics using
blood cell count parameters. The aim was to assess the value of Matos and Carvalho index (MCI)
in detecting TM from patients presented with microcytic anemia.
PATIENTS AND METHODS: The study was carried out on 171 patients who were diagnosed as
cases of hypochromic microcytic anemia in Kut Hemato‑oncology Center. By Measuring hematological
parameters using five automated red cell discriminative indices (red blood cell (RBC) count, RBC
distribution width, Shine and Lal index, MCI index, and Mentzer index [MI]) with measuring hemoglobin
(Hb) A2 levels using Hb variant B thalassemia short arm program.
RESULTS: Of 171 patients screened for TM, 108 patients were diagnosed as TM by Hb
electrophoresis. Patients with TM presented with the mean age of 25.3 years, while the mean of
age in patients with other LRCI anemia was 6.2 years. RBC count was the best index of correctly
identifi ed patients as 84%, followed by MI and MCI with 74% and 72%, respectively. Furthermore,
the RBC count was the best indicator Youden’s indices (58.2), with high sensitivity for BT (96.3%)
followed by MI with Youden’s index (38). Wide thalassemia mutation play important role in this issue.
CONCLUSION: RBC count are simply accessible and dependable ways for identifying beta
thalassemia trait, but there are no red cells indices and methods have 100% specificity, efficacy,
and sensitivity for the differentiation beta TM from other hypochromic microcytic anemia which may
be due to wide thalassemia mutations.