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Bangladesh Journal of Medical Science Vol. 13 No. 01 January’14

BACKGROUND
Acute promyelocytic leukaemia (APML) is a condi-
tion classically characterised by t(15,17) (q22,q21),
with disseminated intravascular coagulopathy (DIC)
being a major cause of death. Central nervous sys-
tem involvement with APML commonly occurs in
relapse; however, it is rarely seen at presentation,
with only six reported cases in the literature1.
Approximately 20 to 30% of patients died of hemor-
rhage, most often intracerebral in location, either at
presentation or after the initiation of chemothera-
py2,6.  the bleeding diathesis  became a major focus
of research because the outcome of patients with
APL appeared to be more favourable compared  with
that of patients with other subtypes of AML.

CASE REPORT
A 17year male without a significant medical history
was referred to our hospital in January 2013 com-
plaining of repeated seizures for last 2 weeks. Later

on he developed vomiting and photophobia. The
haematological  finding obtained was  leukocyte
count (3,28,000/mm3), hemoglobin (5.6 g/dL),
platelet count(34,000/mm3), and differential white
cell count was  lymphocyte 11%, myelocyte
08%,promyelocyte  41%, myeloblast  40%. Bone
marrow examination revealed more than 70% hyper-
granular promyelocytes with atypical features.
Laboratory tests showed a prolonged prothrombin
time (27 seconds) and activated partial thromboplas-
tin time( 41 seconds), the presence of D-dimer, a fib-
rinogen concentration of  84 mg/dL all of which
were compatible with DIC. The CT scan of brain
revealed two frontal areas of hyperattenuation,
which was suggestive of hemorrhage. The patient
was diagnosed as Acute Promyelocytic Leukemia
with Intracerebral haemorrhage. He was transfused
with fresh frozen plasma. The patient died on the
same day.
Acute promyelocytic leukaemia is frequently associ-

Case report:
Acute Promyelocytic Leukemia in a 17yr Male presenting as Intracerebral Haemorrhage

Rauta S1, Sahoo A K2

ABSTRACT
Acute promyelocytic leukemia (APML), once highly fatal, has emerged as the most curable
subtype of acute myeloid leukemia in adults.  Early mortality most often is due to a severe cat-
astrophic bleeding, often intracerebral in location. Here we report a 17year male patient pre-
sented with status epilepticus having high leukocyte count (3,28,000/mm3) and low platelet
count(34,000/mm3).Peripheral blood & bone marrow was showing good no. of atypical
promyelocytes. CT scan of brain revealed an intracerebral haemorrhage with laboratory profile
of prolonged prothrombin time  and activated partial thromboplastin time, the presence of D-
dimer and decreased fibrinogen concentration. The patient was diagnosed as Acute
Promyelocytic Leukemia with Intracerebral haemorrhage. The patient died on the same day.
APML is the notorious subtype of acute myeloid leukemia which causes fatal intracranial
haemorrhage which has high mortality and morbidity. Clinically significant coagulopathy is
present in 70%– 80% of APML patients at the time of diagnosis.  Early detection and aggres-
sive correction of coagulopathy may prevent the catastrophic event. Prompt image study for
locations and types of ICH can predict outcomes.

Key Words : Acute Promyelocytic Leukemia, DIC, Intracerebral Haemorrhage

1. Rauta S ,Asst. Professor, Dept. of Pathology.
2. Sahoo A K, Asst. Professor Dept. of General Medicine.  Maharajah’s Institute of Medical Sciences
Vizianagaram, Andhra Pradesh 535217 India

Corresponds to: Rauta S, Asst. Professor, Dept. of Pathology. Maharajah’s Institute of Medical Sciences
Vizianagaram, Andhra Pradesh 535217, India

DOI: http://dx.doi.org/10.3329/bjms.v13i1.14482
Bangladesh Journal of Medical Science Vol. 12 No. 05 January '14 Page 88-90

88



ated with clotting abnormalities and carries a high
risk of intracranial haemorrhage7. CNS involvement

in APML is extremely rare at presentation but not
infrequent at relapse, and associated factors include
raised white cell count (>10 x 109/l), prior CNS
haemorrhage, microgranular variant and bcr3
PML/RAR type8,9. Past studies have revealed that
fatal intracranial hemorrhage (40%) is the leading
cause of death from cytotoxic chemotherapy10 ,
although current treatment that combines all-trans-
retinoic acid (ATRA) and conventional chemothera-
py has much improved  the prognosis.
Routine coagulation tests reveal prolongation in the
PT and aPTT, low fibrinogen levels, and elevation in

the D-dimer and fibrin degradation products (FDPs)
in most but not all patients with APL11. These
observations suggests that the bleeding diathesis is
due to disseminated intravascular coagulation.The
pathogenesis  includes  consumptive coagulopathy
and procoagulant activity, fibrinolysis, proteolysis &
increased angiogenesis. The use of ATRA to treat
APL, especially in combination with chemotherapy,
in patients with coagulopathy has improved the sur-
vival rate12-15. Few case supports that high-risk
patients, in particular those who have intracranial
haemorrhage and high white cell count  at  presenta-
tion, should have diagnostic lumbar  puncture per-
formed earlier once the coagulopathy has resolved.
This approach may allow earlier detection and treat-
ment of  occult CNS disease and consequently
reduce the risk of future relapse.

CONCLUSION:
Acute promyelocytic leukemia (APL), once highly
fatal, has emerged as the most curable subtype of
acute myeloid leukemia in adults. Cure is now
expected in 70 to 90% of patients when treatment
includes  ATRA combined with anthracycline based
chemotherapy. Early mortality most often is due to a
severe and often catastrophic bleeding, often intrac-
erebral in location, and remains a major cause of
treatment failure. The most important therapeutic
strategy is early institution of ATRA at the first sus-
picion of the diagnosis (without waiting for genetic
confirmation) and aggressive blood product.

89

Acute Promyelocytic Leukemia  presenting as Intracerebral Haemorrhage.

CT Scan of Brain showing Intracerebral Haemorrhage

Bone Marrow showing good no. of hypergranular 
promyelocytes with prominent nucleoli(Leishman stain)

Bone Marrow showing good no. of hypergranular 
promyelocytes with prominent nucleoli(Leishman stain)



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