Neonatal pancytopenia. Hematology of neonatolgy part

1. Arba Minch University
College Of Medicine And Health Sciences
School Of Nursing
Post Graduate Program On Neonatal Nursing
Course Title : Advanced Neonatal Nursing 1
Individual Assignment On: Pancytopenia
By Sisay Berane ID No. PRMHS /083/15
Submitted To: Instructor Nega D.(Ass . Prof)

2. Introduction
• Pancytopenia refers to a reduction below
normal values of all 3 peripheral blood lineages:
• Leukocytes /white blood cells (leukocytopenia): ,
platelets (thrombocytopenia)., and erythrocytes/
red blood cells (anemia).
• Identifying the etiology of pancytopenia usually
requires microscopic examination of the
peripheral blood smear,
• bone marrow biopsy and aspirate specimens to
assess overall cellularity and morphology

3. Introduction cont..
• Pancytopenia refers to a condition characterized by a
decrease in the number of all three major blood cell types
• It is a rare condition in neonates, but it can have serious
consequences if not promptly diagnosed and managed.
• Pancytopenia can lead to symptoms such as fatigue,
increased susceptibility to infections, and bleeding
tendencies.

4. Introduction cont..
• The prefix "pan-" means "everything," "cyto" means "cell", and
the suffix "penia" means "deficiency."
• The laboratory findings of pancytopenia related to bone marrow
suppression may be caused by :-
A) insufficient production (eg. aplastic anemia)
B) inability of cells to mature (myelodysplasia)
C)replacement of normal bone marrow with fibroisis
(mylelofibrosis)
D) peripheral sequestration that is not related to the bone
marrow (e.g. splenomegaly or hypersplenism).

5. Definition of neonatal pancytopenia
• Pancytopenia is said to be happened in neonates
when :-
hemoglobin is < 13 g/dl in males and < 12 g/dl in
females,
 absolute neutrophil count (ANC) is < 1500
cells/mm3, and
platelet count is < 150 × 103 cells/mm3

6. Defn_ …..
Congenital pancytopenia is a rare and often killer
condition.
Current knowledge of lymphoid and hematopoietic
regulators of human hematopoiesis showed that
these is genetic causes of bone marrow failure
disorders
Epidemiology
The incidence of pancytopenia frequently has a bi-
modal presentation in children and adults in the 3rd
and 4th decades.
Literature has reported a between 1.4 and 2.6 to 1
male to female predominance.

7. Classification of pancytopenia
Constitutuinal /inherited/:-
 decreased marrow production of the 3 major hematopoietic
lineages on an inherited basis
 Acquired pancytopenia:-destruction of hematopoietic progenitors,
disruption of the marrow microenvironment, or
 Immune-mediated suppression of marrow elements due to:
a. Direct cyto-toxic effect on hematopoietic stem cells from a drugs or
chemicals
b. Cell-mediated or antibody-dependent cyto-toxicity, (i.e. genetic
predisposition to bone marrow failure, most cases are idiopathic

8. Causes of pancytopenia
some of the causes pancytopenia :
An autoimmune condition that attacks the bone
marrow
 A viral or bacterial infection
Cancer, chemotherapy, or radiation therapy
 Exposure to toxins such as benzene, arsenic, or
insecticides
Not enough folic acid or vitamin B12 in the
foods …

9. Pathophysiology
Pathophysiology depends on the cause of
pancytopenia:
 The pathophysiology of aplastic anemia is an
autoimmune-mediated T-cell activation
 This leads to the destruction of the hematopoietic
stem cells.
 Bone marrow suppression is also caused by the
direct cytotoxic effects of medications like
methotrexate, anticonvulsants
 chemotherapeutic agents..

10. Pathophysiology cont…
If there is sepsis , pancytopenia happens through
the following mechanisms :
 marrow suppression
 Hypersplenism and
 consumptive coagulopathy
NB:- The above mechanisms usually act in
combination.
 The virus causes pancytopenia through several
mechanisms that modulate the hematopoietic stem
cells.
.

11. Clinical features
 Feeling tired, weak, dizzy, or short of breath
 Frequent fevers or infections
 Pale skin or purple or red dots on the skin
 Bleeding from the gums or nose, blood in bowel
movements or urine, or heavy bleeding from a cut
 Bruising easily, or getting bruises without an
injury

12. Diagnosis
Tests used to diagnose pancytopenia are :
 Blood tests are used to check your child's blood cell
levels.
 Other blood tests are used to find the cause of
pancytopenia.
 A bone marrow biopsy is a procedure to remove a small
sample of bone marrow from your child's bone.

 This procedure is used to check how well your child's
bone marrow is making blood cells.
 The bone marrow sample is also checked for tumors or
anything that prevents the bone marrow from making
healthy cells.

13. DIAGNOSTIC APPROACH TO ANEMIA IN THE NEWBORN
A. The family history should include questions
about anemia, jaundice, gallstones, splenectomy.
B. The obstetric history should be evaluated.
C. The physical examination may reveal an
associated abnormality and provide clues to the
origin of the anemia.
D. Acute blood loss leads to shock, with cyanosis,
poor perfusion, and acidosis.
E. Chronic blood loss produces pallor, but the
infant may exhibit only mild symptoms of
respiratory distress or irritability.

14. DIAGNOSTIC APPROACH TO ANEMIA Cont…
F. Chronic hemolysis is associated with pallor, jaundice, and
hepatosplenomegaly.
G. Complete blood cell count. Of note, a capillary blood Hct is
3.7% to 2.7% higher than a venous Hct. Warming the foot
reduced the difference.
H. Reticulocyte count (elevated with chronic blood loss and
hemolysis, depressed with infection and production defect)
I. Blood smear (Table 45.2)
J. Coombs test and bilirubin level

15. Differential Dx
• A hypocellular bone marrow (frequently associated
with )
• acquired aplastic anemia,
• myelodysplastic syndrome,
• inherited bone marrow failure syndromes such as
fanconi anemia and dyskeratosis congenita
• variety of immunological disorders including
hemophagocytic lymphohistiocytosis.
• Thorough bone marrow analysis is of special
importance for the diagnostic work-up of most
patients

16. Management of Pancytopenia
 Treatment of pancytopenia depends on the
underlying cause and severity of the condition.
 The newborn/ child may need any of the following:
. A blood transfusion:-
 It can help increase red blood cell, white blood cell,
and platelet levels.
This may prevent bleeding or organ damage.
This does not treat pancytopenia.
 A blood transfusion may keep your child safe until
the cause of pancytopenia is known.

17. Management cont…
A stem cell transplant:-
It is a procedure to replace unhealthy stem cells with
healthy cells.
Stem cells are able to become all of the blood cells.
Stem cells can also travel to your child's bone
marrow and can become new bone marrow cells.
 Keep and prevent any means of and cause of
bleeding.
 Providing directed medications
Monitoring vital signs
 Monitoring electrolyte balance

18. General management
Prevent or control bleeding
 Do not give aspirin or NSAIDs
 Use caution with skin and mouth care.
 Apply firm, steady pressure to stop bleeding from a
wound.
 Providing directed medications
 Monitoring vital signs
 Monitoring electrolyte balance

19. general mg cont..
Prevent infections
Wash your hands and your child's hands often.
Use an alcohol-based hand rub if soap and water
are not available.

20. summary
• Pancytopenia is indicated in neonatal when hemoglobin is
< 13 g/dl in males and < 12 g/dl in females, absolute
neutrophil count (ANC) is < 1500 cells/mm3, and platelet
count is < 150 × 103 cells/mm3
• Pancytopenia incidence frequently has a bi-modal
presentation in children and adults in the 3rd and 4th
decades and it can be classified as inherited and acquired.
• Pathophysiology depends on the cause of pancytopenia.

21. Summary cont..
• The main clinical presentations of pancytopenia are:
short of breath, frequent fevers or infections, pale skin or
purple or red dots on the skin, bleeding from the gums or
nose, blood in bowel movements or urine, or heavy
bleeding from a cut, bruising easily, or getting bruises
without an injury
Areas of DDx are : aplastic anemia, myelodysplastic
syndrome, inherited bone marrow failure syndromes such as
Fanconi anemia and dyskeratosis congenita, and a variety of
immunological disorders including hemophagocytic
lymphohistiocytosis are areas of differential diagnosis.
• Pancytopenia can be treated depend on cause but blood
transfusion and stem cell transplant are central treatment
areas..

22. Acknowledgement
It is my deepest gratitude to Arba Minch
University College of Medicine and Health Science
for running this program, and my heartfelt
gratitude goes to instructor Nega D. (Asst.prof.)
for his lecture on course Advanced Neonatal
Nursing I, and for his clear and interactive
lectures, friendly approach, and unchangable
kindness.

23. References
1. Cloherty_and_Stark's_Manual_of_Neonatal_Care_9th_Edition_2023.p
df
2. Nelson Textbook of Pediatric 21e.pdf
3. Approach to pancytopenia: Diagnostic algorithm for clinical
hematologists Jerome Gnanaraja,⁎, Aric Parnesb, Charles W. Francisc,
Ronald S. Goe,
4. Hussain K, Salat MS, Mohammad N, Mughal A, Idrees S, Iqbal J, et al.
Meropenem-induced
5. pancytopenia in a preterm neonate: a case report. Journal of Medical
Case Reports. 2. Goldman FD, Gurel Z, Al-Zubeidi D, Fried AJ, Icardi M,
Song C, Dovat S. Congenital
6. pancytopenia and absence of B lymphocytes in a neonate with a
mutation in the Ikaros gene. Pediatr
7. 3. Erlacher M, Strahm B. Missing Cells: Pathophysiology, Diagnosis, and
Management of (Pan)Cytopenia in Childhood. Frontiers in Pediatrics.
2015; 3.