pancytopenia in general, common causes, plan of treatment, and the process of complete manage severe symptoms

1. Pancytopenia in paediatrics
Dr. Muhammad Isaac m. suliman
MBBS
Portsudan emergency teaching hospital
Sudan
7/Feb/2021

2. overview
• Pancytopenia mean: Reduction in the all major
cellular component of the peripheral blood below
the lower limits
RBCs
WBCs
PLTs
• Reduction in two referee ass Bicytopenia
- Anemia + thrombocytopenia- 77.5%
- Anemia + leukopenia- 17.3%
- Thrompocytopenia + leukopenia- 5.5%

3. Causes
• Reduce production
- Infiltration
- Marrow failure
- Ineffective marrow production
• Peripheral Destruction
- Hypersplenism

4. • Diagnosis made by results of CBC
- Anaemia
- Leukopenia
- Thrombocytopenia

5. Pancytopenia causes in childhood
• Megaloblastic anemia: hypercellular BM
• Aplastic anemia
• brucellosis
• Malignant infiltration: ALL/AML , Hodgkin’s D
• Storage disorder
• Viral infection
• Immune disorders: SLE, Evan’s syndrome,
thymoma
• Other: Anorexia nervosa (hypercellular BM with
fat necrosis)

6. • Aplastic anemia: stem cell disorder, which Bone
marrow stop making cells, common age 7 yrs
- Presentation is with ecchymosis and petechiae
- Lead to anemia, infection, bleeding tendency
- Autoimmune triggered by drug, viruses or
irradiation
- Bone marrow biopsy is diagnostic
- Management is only supportive in asymptomatic
pt also transfusion can solve a lot of complication

7. • Brucellosis : zoonotic infection
pathogen's tropism for central (bone marrow) and
peripheral (spleen) organs of the reticuloendothelial
system (RES).
Causes: ingested raw animal milk and, positive family
history of brucellosis
symptoms and signs included; excessive sweating (68%),
bone aches (62%), chills (55%), arthritis (32%), and
hepatosplenomegaly, petechiea and purpura
a rapid slide serum agglutination test for presence of (B.
melitensis) is diagnostic

8. Common cause in sudan
• A plastic anaemia
• Megaloblastic anaemia
• Visceral leishmaniasis
• Marrow fibrosis
• Malaria
• thalasemias
• ITCP
• Acute leukaemia
• Fanconi anaemia
• Infection (septicaemia, HIV, enteric fever, brucellosis, TB, viral
hepatitis)
• Congestive Splenomegly
• Tropical Splenomegly

9. Present as
• Fever
• Pallor
• Petechial rash
• Bleeding
• Bone pain
• Hepatomegly
• Splenomegly
• lymphadenopathy

10. Approach
History
Clinical examination
Lab evaluation

11. Further evaluation
• Peripheral smear examination
• Bone marrow aspiration
• Bone marrow biopsy
• Other specific invx:
- Serum reticulocyte count
- Serum LFTs
- Bleeding profile
- Coomb’s test
- Serum B12 & folate
- Serum HIV & nucleic acid testing

13. Peripheral smear
• Red cell morphology
 Normocytic normochromic with no anisopoikiloctosis – aplastic
anemia
 Macro ovalocytes with howell jolly bodies – megaloblastic anemias
 Macrocytic – fanconi anemia
• WBCs
 Leucopenia (mostly mature 80%) – aplastic anemia
 Neutrophils present in increased number with toxic granules –
infection
 Hypersegmented neutrophils – megaloblastic anemia
 Blast – leukemia
• Platelets
 Giant platelets - Hypersplenism

14. Anisocytosis & poikilocytosis

15. Bone marrow examination
• Aspiration and biopsy
Hypocellular
Cellular
Bone marrow filtration

18. Plane of treatment
• Transfusions of RBCs and Platelets as needed
• AB Prophylaxes
• Prophylactic B12 vitamin
• Corticosteroids
• Androgenic therapy
• Bone marrow transplant from an HLA-
compatible donor is curable.

19. Any Questions?

20. Problem solving
• infant was delivered via spontaneous vaginal delivery at 384 weeks’
gestational age. She was born following in vitro fertilization to a 31-
year-old primigravida mother known to have hypothyroidism. There
was no reported consanguinity between the parents. Maternal
toxoplasmosis, other (syphilis, varicella-zoster, parvovirus B19),
rubella, cytomegalovirus and herpes infections (TORCH) serology
was negative and her blood count was normal. Fetal ultrasound
revealed an absent right kidney but otherwise normal anatomy.
Integrated prenatal screen was normal. The mother received one
prophylactic dose of penicillin G for group B Streptococcus-positive
status. At delivery, the infant had normal vital signs and no
dysmorphic features; however, she was pale, with a ‘blueberry’ rash
(palpable purpura-petechiae) covering her entire body (Figure 1)

22. • The first complete blood count performed after birth revealed a
hemoglobin level of 5.2 g/L, platelet count of 3×109/L and a
relatively low white blood cell count (6.1×109/L) but normal
neutrophil count (1.6×109/L). She immediately received platelet and
red blood cell transfusions. Five days later, she developed severe,
persistent. A fundoscopic examination revealed bilateral retinal
hemorrhages. Magnetic resonance imaging of the brain revealed
multiple small cortical petechial hemorrhages
Q:
1. What’s the diagnosis?
2. How to confirm ur diagnosis ?
3. Plane of management ?

23. THANX FOR UR listening!