Concurrent intracranial chloromas and leukemia cutis in chronic myeloid leukemia
V O L .
cutis in chronic myeloid leukemia Concurrent intracranial chloromas and leukemia
Ateeque Ahmed Khan 0
0 Civil Hospital & Dow University of Health Sciences (DUHS) , Karachi , Pakistan
CONCURRENT INTRACRANIAL CHLOROMAS AND
LEUKEMIA CUTIS IN CHRONIC MYELOID LEUKEMIA
Ateeque Ahmed Khan1, Mahnoor Hafeez1
1Department of Radiology, Civil Hospital & Dow University of Health Sciences (DUHS), Karachi, Pakistan.
Address correspondence to 2- Dr. Mahnoor Hafeez; Resident PGY-IV, ;
Date of submission: March 29, 2016 Date of revision: June 20, 2016 Date of acceptance: June 27, 2016
Granulocytic sarcoma (chloroma) is an extramedullary solid tumor composed of immature white blood cells. Most
common involvement sites are bone, periostium, soft tissue, lymph nodes and skin. Intracranial granulocytic sarcoma
rarely occurs in meningeal or parenchymal form1. We report the of concurrent occurrence of intracranial chloromas
and leukemia cutis in 26 years old male patient, known case of Chronic Myeloid Leukemia for 3 years [+ve
Philadelphia (Ph) chromosome in bone marrow cells] who now presented to the Civil Hospital Karachi with continuous
headache and multiple cutaneous nodules all over the body for last 2 months. The CT exam of the Head revealed
multiple hyper dense extra-axial nodular masses (with mean value of 48 HU) which showed vivid enhancement on post
contrast images, along with multiple subcutaneous enhancing nodules seen in the scalp and maxillofacial region. The
CSF cytological examination confirmed presence of immature leukemic cells in the subarachnoid space. The
subcutaneous chest nodule histopathology showed granulocytic sarcoma. To the best of authors’ knowledge, the
concurrent occurrence of granulocytic sarcomas at different locations in a single patient with Chronic Myeloid
Leukemia has not been reported earlier in the literature.
Chronic Myeloid Leukemia; CML; CT; HU; leukemia cutis; intracranial chloroma; Granulocytic sarcoma.
Chronic myelogenous (or myeloid or myelocytic)
leukemia (CML) is a cancer of the white blood cells. It
is a form of leukemia characterized by the increased
and unregulated growth of predominantly myeloid cells
in the bone marrow and the accumulation of these cells
in the blood. It is a type of myeloproliferative disease
associated with a characteristic chromosomal
translocation called the Philadelphia chromosome.
CML is now largely treated with tyrosine kinase
inhibitors (TKIs) –imatinib which have led to
dramatically improved long-term survival rates. CML
accounts for 15-20% of all adult leukemia. Information
on CML incidence and prevalence is scare, as CML is a
rare disease 2. CML is often divided into three phases
based on clinical characteristics and laboratory
findings. It typically begins in the chronic phase, and
over the course of several years progresses to an
accelerated phase and ultimately to a blast crisis. Blast
crisis clinically behaves like an acute leukemia. Blast
crisis is diagnosed if any of the following are present in
a patient with CML: >20% myeloblasts or lymphoblasts
in the blood or bone marrow or the Development of a
chloroma/ Granulocytic sarcoma3-4. Granulocytic
sarcoma is a extramedullary proliferation of leukemic
cells. It a manifestation of acute myeloid leukemia
(AML), chronic myeloid leukemia and other
myeloproliferative disorders. They occur in 0.5-1.8% of
patients with chronic myelogenous leukemia5. Most
common involvement sites are bone, periostium, soft
tissue, lymph nodes and skin. Intracranial granulocytic
sarcoma rarely occurs in meningeal or parenchymal
form1. CT and MRI have been the most commonly
utilized imaging modalities for assessment of CNS
myeloid sarcoma. These sarcomas are very sensitive to
focal irradiation or chemotherapy5. To the best of
authors’ knowledge, the concurrent occurrence of
multiple cutaneous granulocytic sarcomas as well as
multiple intracranial granulocytic sarcomas have not
been reported earlier in the literature. We hereby
present concurrent intracranial chloromas and
leukemia cutis in chronic myeloid leukemia as the first
P A K I S T A N J O U R N A L O F N E U R O L O G I C A L S C I E N C E S
A 26-year-old Asian male, resident of thatta presented to the Civil Hospital 3 years back in August 2013 with
complaints of chronic fever, abdominal pain and swelling. Fever was continuous, low grade and not relived by
antipyretics. There was no significant family history. He underwent karyotype analysis as well as bone marrow trephine
biopsy from right iliac blade. Karyotyping showed reciprocal translocation of chromosome segment between
chromosomes 9 and 22. The biopsy revealed Philadelphia (Ph) chromosome in bone marrow cells and was labeled as
chronic myeloid leukemia in chronic phase He was kept on tab Gleevec (Imatinib mesylate) PO 400 mg per day. On
which his symptoms were well controlled. Meanwhile, the patient was lost on follow up visits due to personal issues
and now he presented again to the Civil Hospital in September 2016 with complaints of innumerable cutaneous
nodules all over the body for last 2 months and continuous severe headache for 25 days, not relieved by anlagesics.
There was no history of trauma. On admission, laboratory investigations showed hemoglobin 8 g/dl, mean corpuscular
volume 86.9fl, platelet count 132 × 109/L and white blood cell count 134.6 × 109/L, with monocytes(raised) 12%,
neutrophils 66% and lymphocytes 11%. The peripheral smear revealed promyelocytes, myeloblasts, myelocytes,
metamyelocytes, nucleated red blood cells are seen with basophilia and Leukoerythoblastic picture. Ultrasound
exam revealed massive splenomegaly measuring
22.0cm. Computed tomography of the Head was
performed on 16 slice Toshiba Activion Spiral CT scanner
with dual head power injector and scanning parameters
of 120 KV, 150mA and 5mm slice thickness; images were
obtained in Pre and Post Contrast phases in multiple
planes and viewed at appropriate window settings.
There were multiple well de ned intracranial con uent
as well as discrete hyperdense extra-axial masses at right
frontoparietal region, right sylvian ssure and at right
para falcine region, also along the Tentorium cerebelli;
Largest measures 8mm in width, they are hyper dense to
matter with mean value of 48 HU which demonstrated
homogenous enhancement with post contrast mean
attenuation value of 69HU. No adjacent parenchymal
edema was seen. Bilateral orbital region was typically
spared. Visualized bones appeared unremarkable.
There were multiple subcutaneous enhancing nodules
seen in scalp and maxillofacial region. Largest along
the left maxillary labial fold measures 29x16mm. The
CSF cytology via lumbar puncture confirms infiltration of
myelocytes and myeloblasts in the subarachnoid
space. The tru-cut biopsy of the largest subcutaneous
nodule at left anterior chest wall measuring 40x40mm
showed blast transformation to acute myeloid leukemia
and diffuse infiltration of myeloid blast cells in the
dermis and subcutaneous tissue.
Our case describes 26 years old male- a known case of
Chronic Myeloid Leukemic for last 3 years, and was
kept on glevaac and now presented with continuous
severe headache and multiple nodules all over the
body for last 2 months. Our clinical suspicion was brain
leukemia. His CT of the head demonstrated the
presence of multiple hyper dense enhancing intra
cranial extra-axial nodular masses, confirmed as
immature leukemic cells on CSF cytologic examination.
The investigations revealed blast crises of CML and
concurrent occurrence of granulocytic sarcoma in skin
and intracranial space. O'Brien CE et al in 20116 at
Washington, DC presented a case report of intracranial
extra axial Granulocytic Sarcoma on CT in a34-year-old
chronic myelogenous leukemic patient with blast crisis
mimicking as subdural hematoma -comparable to our
case. In 2015, Cervantes GM et al7 studied summary of
clinical findings, CT and MR features of intracranial
myeloid sarcomas (MS) in 21 reported cases
presenting with AML from 1971 to 2014. 12 out of a
total of 24 lesions were assessed with CT. Among
these, 11 lesions (91%) appeared hyperdense on
non-contrast CT. Out of all 11 hyperdense lesions, six
lesions were intra-axial and five extra-axial. 91% lesions
exhibited avid homogeneous enhancement. The CT
findings of these case reports were compatible to our
In 2011, Out of 60 patients of chronic myeloid
leukaemia studied over 21 months at Rawalpindi by
Ahmed S. et al 8, 6(10%) cases of granulocytic
sarcoma were encountered, sites of extramedullary
disease were skin, breast, spinal canal and lymph
nodes and none had intracranial involvement. Kanade
U et al9 reported a case of chronic myelogenous
leukemia cutis in a 70-year-old male patient presented
with nonspecific abdominal discomfort and multiple
papular and nodular skin lesions all over the body for 6
months. Aspiration cytology smears from cutaneous
nodule revealed variable mixture of mature and
immature cells of granulocytic series – again
comparable to our case. To the best of authors’
knowledge, the concurrent occurrence of multiple
cutaneous granulocytic sarcomas as well as multiple
intracranial granulocytic sarcomas in chronic
myelogenous leukemia have not been reported earlier
in the literature. The patient was referred to the
oncology department for AML induction chemotherapy.
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