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Original Article

Pancytopenia in children: A 6-year spectrum of patients admitted


to Pediatric Department of Rehman Medical Institute, Peshawar
Anwar Zeb Jan1, Bakhtyar Zahid2, Samreen Ahmad3, Zahid Gul4
ABSTRACT
Objective: To determine the various spectrum of pancytopenia with its frequency on the basis of bone
marrow examination in children from 6 months to 14 years.
Methods: A retrospective descriptive study was carried out at Department of Pediatric Rehman Medical
Institute Peshawar from January 2006 to December 2012. A total of 205 patient’s age between 6 months
and 14 years, fulfilling the inclusion and exclusion criteria were included in the study. Complete blood
count, peripheral smear, bone marrow examination and Serum vitamin B12 level was done in all the cases.
Results: Out of 14642 patients admitted to the Pediatric department during the study period, 205 (1.4%)
patients were pancytopenic on their peripheral blood smear. Male outnumbered female with a ratio of
1.8:1. 42.5% of the patients were in the age group of 1 month to 5 years. Common etiological pattern
identified were Aplastic anemia 58(28.3%), Hematological malignancies 49 (23.9%), megaloblastic anemia
40 (19.5%), idiopathic thrombocytopenic purpura 16 (7.8%), iron deficiency anemia 9 (4.4%), hemolytic
anemia 7 (3.41%), Visceral leishmaniasis 6 (2.93%), hypersplenism 5 (2.44%), malaria 5 (2.44%), anemia of
chronic disorder 4 (1.95%), Myelodisplastic syndrome 3 (1.46%), Niemen pick disease 2 (0.97%) and Gaucher
disease in 1(0.49%). Common clinical presentations were fever, pallor, body aches, petechial hemorrhages
and epistaxis.
Conclusion: Pancytopenia is one of the importance occurrences in pediatric patients. Acute leukemia
and bone marrow failure are the most common causes yet megaloblastic anemia and infections are the
treatable and reversible causes of pancytopenia.
KEY WORDS: Anemia, Bone Marrow, Megaloblastic, Pancytopenia.
doi: http://dx.doi.org/10.12669/pjms.295.3865
How to cite this:
Jan AZ, Zahid B, Ahmad S, Gul Z. Pancytopenia in children: A 6-year spectrum of patients admitted to Pediatric Department of
Rehman Medical Institute, Peshawar. Pak J Med Sci 2013;29(5):1153-1157. doi: http://dx.doi.org/10.12669/pjms.295.3865
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0),
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

INTRODUCTION prevailing when the hemoglobin (Hb) <10g%,


absolute neutrophil count (ANC) <1.5*109/L,
Pancytopenia is a malady in which there is platelet count <100*109/L. The pancytopenia
lessening of all the three cellular elements of blood; was labeled as severe if patient had two or more
of the following: Hb <7 gm%, ANC <0.5*109/L,
1. Dr. Anwar Zeb Jan, MRCP,
2. Dr. Bakhtyar Zahid, MRCPCH, and platelet count <20*109/L.1 In pancytopenia
3. Dr. Samreen Ahmad, FCPS, the marrow is customarily hypocellular as a
4. Dr. Zahid Gul, MCPS (Trainee),
1-4: Pediatric Department Rehman Medical Institute,
result of primary production defects, it can be
Peshawar, Pakistan. due to diminution of hemopoitic cell production,
Correspondence: ineffective haemopoiesis or may be due to peripheral
devastation of cells. The production of hemopoitic
Dr. Anwar Zeb Jan,
Clinic # 30 First Floor, 5/B-2 Phase-5, cell can be prejudiced in the bone marrow either
Hayatabad, Peshawar, Pakistan. by infections, toxins, and malignant cell infiltration
Email: dranwarzebrmi@yahoo.com
leading to hypocellular marrow. Ineffectual
* Received for Publication: May 24, 2013 hematopoiesis and dysplasia, maturation arrest
* Accepted for Publication:* July 8, 2013 of all the cell lines and peripheral sequestration of

Pak J Med Sci 2013 Vol. 29 No. 5 www.pjms.com.pk 1153


Anwar Zeb Jan et al.

blood cells or peripheral destruction of all blood condition like megaloblastic anemia whose picture
cells lineage can also be the cause of pancytopenia.2-4 of presentation is very drastic but can be easily
Pancytopenia is a common hematological problem managed.
with an extensive differential diagnosis, and still
the optimal diagnostic approach to pancytopenia METHODS
remains undefined.5-7 Pancytopenia itself is not a This was a retrospective descriptive study carried
disease but actually a triad of findings that may out at the Department of Pediatric of Rehman Med-
result from a number of disease processes associated ical Institute Peshawar from 01st April to 15th May,
with bone marrow both affecting it primarily 2013and the data taken was of the last 6 years i.e.
or secondarily and resulting in pancytopenia.8 from 01 January 2006 – 31 December 2011.A total
Most often the Pancytopenia is associated with of 205 cases presenting with pancytopenia on pe-
organomegaly and lymphadenopathy usually ripheral blood smear were included in the study,
suggests the possibility of malignancies or bone fulfilling the inclusion and exclusion criteria.
marrow failure syndromes but there are a number The inclusion criteria for the study were, children
of other causes which can present in the similar way in the age group 6 months to 14 years admitted in
and are very easily treatable.9 the hospital and who had pancytopenia on their pe-
Megaloblastic anemia has been known as a clinical ripheral blood smear. Pancytopenia was defined as
entity for over a century, the first clinical case of hemoglobin (Hb) <10g%, absolute neutrophil count
pernicious anemia, which is one of the known (ANC) <1.5*109/L, platelet count <100*109/L. The
causes of megaloblastic anemia, has been attributed pancytopenia was labeled as severe if patient had
to Thomas Addison in 1849.10 Megaloblastic anemia two or more of the following: Hb <7 gm%, ANC
results from abnormal maturation of hematopoietic <0.5*109/L, and platelet count <20*109/L.20
cells due to faulty DNA synthesis, the two vitamins All those children who had received blood trans-
namely vitamin B12 and folic acid are essential fusion were excluded from the study. Detailed his-
for DNA biosynthesis and the deficiency of either tory was taken including dietary intake, ingestion
of these vitamin can result in asynchrony in the of any drugs, worm’s infestation, and loss of blood
maturation of nucleus and cytoplasm of rapidly and duration of onset of anemia.
regenerating cells.11,12 Other than megaloblastic The patient fulfilling the inclusion criteria at the
anemia, deficiency of these hematopoietic time of admission had their complete blood count
micronutrients in children has been incriminated to (including Hemoglobin, total Leukocytes count
cause neuro-development dysfunction, abnormal Differential leukocytes platelet count and ANC),
movements and failure to thrive.13 Peripheral smear, serum vitamin B12 and bone
Acquired aplastic anemia usually has an marrow aspiration done. Bone marrow biopsy
autoimmune basis that is diminution of the was done in cases where bone marrow aspiration
hematopoietic stem cells by direct toxicity mostly was inconclusive. Ethical approval was obtained
due to radiations, drugs, chemicals and viruses.14 from the RMI Ethical Review Board regarding data
Anemia of chronic disease is a mild to moderate collection and use for research purposes.
anemia that occurs mostly in infections and
inflammatory disorders15 and is frequently found RESULTS
in chronic kidney insufficiency, dialysis patients, Out of 14642 patients admitted to Department
congestive heart failure and renal transplantation.16 of Pediatrics, 205(1.4%) patients presented with
Anemia can also present in patients with newly pancytopenia. Out of 205 patients, 133 (64.87%)
diagnosed childhood acute lymphoblastic leukemia were males and 72 (35.13%) females, with male to
(ALL).17 Being the most common pediatric female ratio of 1.84:1 (Table-I), their ages ranged
malignancies, ALL represents 25% of all childhood
Table-I: Distribution of patient
malignancies and approximately 75% of all cases of
according to Gender and Age.
childhood leukemia.18
Bone marrow play a vital role in understanding Age Male Female Total
(n-133) (n-72) (n-205)
the etiology of pancytopenia19, in time recognition
of the underlying pathology will not only have 6 months – 5 years 57 30 87 (42.44%)
an impact on the mortality and morbidity of the 6 years – 10 years 46 26 72 (35.12%)
vulnerable pediatric patients but will also help 11 years – 14 years 30 16 46 (22.44%)
us treat the most simple and easily treatable Total 133 72 205 (100%)

1154 Pak J Med Sci 2013 Vol. 29 No. 5 www.pjms.com.pk


Pancytopenia in children

Table-II: Etiological Pattern of Pancytopenia. Table-III: Clinical feature of pancytopenia


Etiology No. of cases Percentage at presentation (n=205).
(n-205) Clinical Feature No. of cases Percentage
(n-205)
Aplastic anemia 58 28.3
Leukemia’s 49 23.9 Pallor 170 82.92
Megaloblastic Anemia 40 19.51 Fever 135 62.85
Idiopathic thrombocytopenia 16 7.80 Bruises 128 62.44
purpura Petechial hemorrhage 40 19.51
Iron deficiency anemia 9 4.4 Malena 30 14.63
Visceral leishmaniasis 6 2.93 Hematemesis 20 9.75
Anemia of chronic disorder 4 1.95 Joint/leg pain 60 29.27
Hypersplenism 5 2.44 Bleeding from gums 50 24.39
Malaria 5 2.44 Epistaxis 60 29.27
Hemolytic anemia 7 3.41 Hematuria 25 12.19
Myelodisplastic syndrome 3 1.46 A patient having more than one clinical feature is counted
in each category. Hence the sum may be more than the
Gaucher disease 1 0.49
total number of cases in the study.
Niemen pick disease 2 0.97
unexplained pallor. In our study the frequency of
from one month to 14 years. Maximum number Pancytopenia was 1.4%, this frequency in other
of patients 87 (42.44%) were in the age group of 6 studies is quite variable. According to a study
month to 5 years, followed by 72 (35.13%) in the conducted in Peshawar in 2000, they reported it to
6 to 10 years age group while minimum number be 0.8%21, while a study done by Shazia Memon in
46 (22.43%) were those exceeding 11 years of age Jomshoro reported it to be 3.57%22, while Adil et al23
(Table-I), all age group had a male predominance. and Kanchanalak et al24 reported it to be 12.6% and
The most common symptom was pallor in 170 1.2% respectively.
(82.92%) cases and fever in 135 (62.85%) which In our study male dominated female in all the
was often prolonged for weeks, other symptoms age group with male to female ratio of 1.8:1, other
included bruises, epistaxis, malena, petechial studies done also had the some finding with male
hemorrhages, hematuria and joint pains (Table-II) dominating female, a study from Peshawar done by
Considering the etiological pattern of all the Afzal Khan25 reported the male to female ratio of
205 cases that were included in the study due to 1.5:1, while a study done by Amieleena C et al9 and
pancytopenia, Aplastic anemia 58 (28.3%) was the Goel RG et al26 reported the male to female ratio of
most common cause of pancytopenia followed by 1.64:1 and 1.76:1 respectively.
leukemia 49 (23.9%) while megaloblastic anemia In our study many disease entities other than
was found in 40 (19.51%) of cases followed by malignancies, significantly Aplastic anemia,
other less common problems like idiopathic Megaloblastic anemia and Infectious causes
thrombocytopenic purpura 16 (7.80%) iron emerged as recognizable cause of pancytopenia.
deficiency anemia 9 (4.4%), Visceral leishmaniasis Aplastic anemia was the most common cause
6 (2.93%), anemia of chronic disorder 4 (1.95%) and of pancytopenia diagnosed on bone marrow
malaria was found in 5 (2.44%) of cases (Table-III). accounting for 58 (28.3%) of cases. Though aplastic
A patient having more than one clinical feature anemia has a pattern of geographical variation
is counted in each category. Hence the sum may be opposite to that of leukemia, with higher incidence
more than the total number of cases in the study. in the developing countries than the developed
one.27 The studies done in European countries
DISCUSSION
indicate the annual incidence of 2 new cases per
Pancytopenia is condition in which there is million of population.28 Studies done in China29 and
reduction of all the 3 peripheral blood lineage, Thailand30 reported the frequency of aplastic anemia
it’s not an uncommon hematological problem to be three folds to that of the western countries of
countered in clinical practice and must be suspected the world. The studies done nationally reported,
on clinical grounds when a patient presents with Afzal Khan25 from Peshawar, reported the aplastic

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Anwar Zeb Jan et al.

anemia to be the most common disorder with a CONCLUSION


frequency of 40 (20.2%) cases out of 198 cases that
were included in the study while Shazia Memon22 Pancytopenia is one of the importance
from Hyderabad reported the incidence of Aplastic occurrences in pediatric patients. Acute leukemia
anemia in 55 (23.9%) cases out of 230 cases included and bone marrow failure are the most common
in study. causes yet megaloblastic anemia and infections are
Another common etiological factor was nutri- the treatable and reversible causes of pancytopenia.
tional deficiency anemia, especially megaloblastic ACKNOWLEDGMENT
anemia. It accounted for 40 (19.51%) patients pre-
senting with pancytopenia. Megaloblastic anemia The authors wish to thank Prof. Mohammad
due to vitamin B12 deficiency is well recognizable Rehman (Chairman Rehman Medical Institute
cause of pancytopenia. Various study reported the and Head of Cardiothoracic Surgery) for his kind
range of pancytopenia in megaloblastic anemia to permission to access the data enabling us to publish
be varying from 11-47%.8 A study done in India this paper and Professor Dr. Mehr Taj Roghani Head
by Bhatanger31, including 109 children in study of Pediatrics Department for her kind assessment
presenting as pancytopenia the incidence of mega- and supervision. We also thank Prof. Dr. Iftikhar
loblastic anemia was 28.4%. Study of 200 cases by Qayyum for helping us in the analysis of data.
Khunger32 reported megaloblastic anemia in 72% Source of funding: Dr. Anwar Zeb Jan.
of cases. Other studies done nationally and inter-
nationally reported the incidence of megaloblastic Conflict of interest: There is no conflict of interest.
anemia to be, Shazia et al22 including 230 children
in the study reported the incidence of megaloblastic REFERENCES
anemia in 40 (17.39%) cases. 11% in a study done be 1. Frank F, Collin C, David P, Byran R, Editor. De Gruchey’s
Gomber et al33 while it has reported to be 47% in a clinical hematology in medical practice. 5thed. Blackwell:
Berlin; 2004:1199.
study done by Mubarik et al34 while Sarode et al35
2. Kar M, Ghosh A. Pancytopenia. J Indian Acad Clin Med.
from India reported the incidence of 80.5%. 2002;3:29-34.
In our study the incidence of Hematological 3. Lee GR, Bithell TC, Forester J, Athens JW, Lukens JN, editors.
malignancies was 49 (23.9%) cases, other studies Wintrobe’s Clinical Hematology. 10th ed. Philadelphia: Lee
done reported the incidence of hematological ma- & Febiger, 1999:1969-1989.
4. Rodak RF. Hematology Clinical Principles and Applications.
lignancies. In our study ALL 24 (11.7%) was the 2nd ed. Philadelphia: W.B. Saunders Company, 2002:63-94.
most common malignant condition presenting as 5. Rehman H, Mohammad F, Faiz M. Clinical presentation of
Pancytopenia followed by AML 16 (7.80%) and the pancytopenia in children under 15 years of age. J Postgrad
least common was CML 9 (4.4%), this finding of Med Inst. 2003;17:46-51.
6. Savage DG, Allen RH, Gangaidzo IT. Pancytopenia in
our study is almost in consistent with a study done
Zimbabwe. Am J Med Sci. 1999;317:22-32.
including 230 cases22 of which 40 cases presenting 7. Osama I, Haider ZB, Anwer F, Nisar H. Patterns of
with pancytopenia were malignant condition, the pancytopenia patients in a general medical ward and
ALL 20 (8.69%) was the most common hemato- a proposed diagnostic ap-proach. J Ayub Med Coll
logical malignancy presenting as pancytopenia fol- Abbottabad. 2004;16:8-13.
8. Niazi N, Raziq F. The incidence of underlying pathology in
lowed by AML 5(2.17%) and CML 2 (0.89%). Afzal pancytopenia - An experience of 89 cases. J Postgrad Med
Khan from Peshawar reported the malignant condi- Inst. 2004;18:76-79.
tion to be one of the most common cause present- 9. Ameileena C, Vipan C, Anubhava P, Harish C. Clinico-
ing as pancytopenia reporting it in 11.3% of cases25, aetiological profile of pancytopenia in pediatric practice.
JIACM 2012;13(4):282-5.
other study done in Islamabad reported the similar
10. Addison T. anemia-disease of the suprarenal capsules.
results while a study done in USA reported the in- London Med Gazette. 1849;43:517-518.
cidence of hematological malignancy in 4.5% cases 11. Antony AC. Megaloblastic anemia in: Hoffman R, Benz
per 100,000 children. EJ, Shatil SJ, Furie B, Cothen HJ, Silberstein LE, et al.
Idiopathic thrombocytopenic purpura accounted Hematology basic principles and practice 4th ed. Edinburgh:
Churchil Livingstone; 2005:519-556.
for 16(7.8%) of cases, being one of the common he- 12. Carmel R. Megaloblastic anemias; disorders of impaired
matological problem in our study. It is also one of DNA synthesis. In: Greer JP, Foerster J, Lukens JN, Rodgers
the commonest cause of purpura. Other studies36 GM, Paraskevas F, glader B (eds). Wintrobe’s clinical
have shown 32% to 48% frequency. While study hematology. 11th ed. Philadelphia: Lippincot Williams and
Wilkins; 2004:1367-1395.
done in Peshawar25 by Afzal Khan, reported it to be
13. Jagdish C. Megaloblastic anemia: back in focus. Indian J
15.7%. Pediatr. 2010;77:795-799.

1156 Pak J Med Sci 2013 Vol. 29 No. 5 www.pjms.com.pk


Pancytopenia in children

14. Gaman A, Gaman G, Bold A. Acquired aplastic anaemia: 26. Goel RG, Bhan MK, Azany S. A study of severe anemia
correlation between etiology, pathophysiology, bone in hospitalized children in Afghanistan. Indian Pediatr.
marrow histology and prognosis factors. Rom J Morphol 1981;18:643-646.
Embryol. 2009;50:669–674. 27. Issaragrisil S, Leaverton PE, Chansung K, Thamprasit T,
15. Matsumura I, Kanakura Y. Pathogenesis of anaemia of Porapakham Y, Vannasaeng S, et al. Regional patterns in
chronic disease. Nippon Rinsho. 2008;66:535–539. the incidence of aplastic anemia in Thailand. The Aplastic
16. Silverberg DS, Wexler D, Blum B, Iaina A. Anaemia in Anemia Study Group. Am J Hematol. 1999;61:164-168.
chronic kidney disease and congestive heart failure. Blood 28. Young NS. Hematopoietic cell destruction by immune
Purif. 2003;21(1):124–130. mechanisms in acquired aplastic anemia. Semin Hematol.
17. Teuffel O, Stanulla M, Cario G, Ludwig WD, Rottgers S, 2000;37:3-14.
Schafer BW, et al. Anaemia and survival in childhood 29. Yang C, Zhang X. Incidence survey of aplastic anemia in
acute lymphoblastic leukemia. Haematologica. China. Chin Med Sci J. 1991;6:203-207.
2008;93:1652–1657. 30. Issaragrisil S, Chansung K, Kaufman DW, Sirijirachai J,
18. Rana ZA, Rabbani MW, Sheikh MA, Khan AA. Outcome of Thamprasit T, Young NS. Aplastic anemia in rural Thailand:
childhood acute lymphoblastic leukaemia after induction its association with grain farming and pesticide exposure.
therapy-3 years’ experience at a single paediatric oncology Aplastic Anemia Study Group. Am J Public Health.
centre. J Ayub Med Coll Abbottabad. 2009;21(4):150-153. 1997;87:1551-1554.
19. De Benoist B, McLean E, Egli I, Cogswell M, (Eds). 31. Bhatnagar SK, Chandra J, Narayan S, Sharma S, Singh V,
Worldwide prevalence of anaemia 1993–2005: WHO global Dutta AK. Pancytopenia in children: etiological profile. J
database on anaemia. Geneva: WHO; 2008. Trop Pediatr. 2005;51:236-239.
20. Alter BP. Bone marrow failure syndromes in children. 32. Khunger JM, Arulselvi S, Sharma U, Rangas S, Talib VH.
Pediatr Clin North Am. 2002;49:973-988. Pancytopenia a clinicohaematological study of 200 cases
21. Habib R, Fazil M, Khan FM. The etiological pattern of .Indian J Pathol Microbiol. 2002;45:475-479.
pancytopenia in children upto 15 years. Pak Armed Forces 33. Gomber S, Kela K, Dhingra N. Clinico-hematological profile
Med J. 2003;53:183-187. of megaloblastic anemia. Indian Pediatr. 1998;35:55-58.
22. Shazia M, Salma S, Akbar N. Etioloigcal spectrum of 34. Mukiibi JM, Makumbi FA, Gwanzura C. Megaloblastic
pancytopenia based in bone Marrow examination in anemia in Zimbabwe: Spectrum of clinical and hematological
children. J Coll Physicians Surg Pak. 2008;18(3):163-167. manifestations. East Afr Med J. 1992;69:83-88.
23. Adil SN, Burney IA, Kakepoto GN, Khurshid M. 35. Sarode R, Garewal G, Marwaha N. Pancytopenia in
Epidemiological features of aplastic anemia in Pakistan. J nutritional megaloblastic anemia. A study from north-west
Pak Med Assoc. 2001;51:443-445. India. Trop Geogr Med. 1989;41:331-336.
24. Ghorpade K, Baldota S. Pancytopenia - its causes in Bombay. 36. Ng Sc, Kuperan P, Chans KS, Bosco J, Chan GL. Anemia- a
J JJ Group Hosp Grant Med Coll. 1991;33:30-32. review from university hospital, Kuala Lumpur. Ann Acad
25. Afzal K, Muhammad A, Taj AK, Arshia M. Pattern Med Sing. 1988;17:261-264.
of hematological disease in hospitalized pediatric
patients based on bone marrow examination. JPMI.
2008;22(3):196-200.

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