Journal of Cancer Therapy, 2013, 4, 1049-1051
http://dx.doi.org/10.4236/jct.2013.46118 Published Online August 2013 (http://www.scirp.org/journal/jct) 1049
Simultaneous Diagnosis of Myeloid Sarcoma of the Jaw
and Mycobacterium tuberculosis Infection
Luís Arthur Flores Pelloso1,2, Sandra Serson Rohr1, Mihoko Yamamoto1,
Maria de Lourdes L. F. Chauffaille1
1Section of Hematology and Blood Transfusion, Department of Clinical and Experimental Oncology, Federal University of São
Paulo (UNIFESP-EPM), São Paulo, Brazil; 2PPD, Pharmaceutical Product Development, Inc., Pharmacovigilance, São Paulo, Brazil.
Email: chauffaille@unifesp.br
Received May 15th, 2013; revised June 17th, 2013; accepted June 24th, 2013
Copyright © 2013 Luís Arthur Flores Pelloso et al. This is an open access article distributed under the Creative Commons Attribu-
tion License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
cited.
ABSTRACT
Granulocytic or myeloid sarcoma (MS) is a rare neoplastic condition consisting of a tumor mass of myeloid blasts with
or without maturation occurring at an anatomical site other than the bone marrow the association between tuberculosis
and MS is extremely rare. A 21-year-old female patient presented cough, sore throat and a suppurative swollen gum for
10 days prior to hospital admissio n. Physical examination revealed moderate pallor and swollen inferior gum. CBC re-
vealed Hb 6.5 g/dL, hematocrit 18.4% MCV 97 fL MCH 34 pg, WBC 18.5 109/µL (1 My/3 Bt/69 Sg/1 Eo/0 Ba/20
Ly/6 Mo), Platelets 43 109/µL. The peripheral blood smear presented with 3% blast cells (type 1) and granulocytic
dysplasia. Bone marrow biopsy show ed 100% cellularity. 50% of cells were fr om granulocytic precursors, d iagnosis of
granulocytic sarcoma. The diagnosis of AML was established: granulocytic sarcoma with massive gum infiltration
(immature granulocytic cells) and 10% of blasts in bone marrow. The patient received induction chemotherapy (3 + 7
daunorubicin 90 mg/m2), and gum tissue culture was positive for Mycobacterium tuberculosis. Simultaneously, a qRT-
PCR test confirmed the same bacteria in the gum tissue. Patient treated with isoniazid, rifampicin, pyrazinamide and
ethambutol ciprofloxacin and amikacin). Remission was achieved and the patient was submitted for consolidation/ in-
tensification (HiDAC x3) schema and referred to allogeneic HSCT. After induction and full hematological recovery
there was no further evidence or recurrence of fever and lytic lesions. Currently patient is under CR and ling follow up
(48 mont hs) did not show recurrence of eit her AML or tuberculosi s .
Keywords: Myeloid Sarcoma; Mycobacterium tuberculosis; Granulocytic Sarcoma
1. Introduction
Granulocytic or myeloid sarcoma (MS) is a rare neo-
plastic condition consisting of a tumor mass of myeloid
blasts with or without maturation occurring at an ana-
tomical site other than the bone marrow [1]. MS may
develop as de novo or simultaneously to acute myeloid
leukemia (AML), myeloproliferative neoplasm (MPN) or
myelodysplastic syndrome (MDS) [1]. Hence, MS may
be the first manifestation of AML and precede it by months
or years, or equally represent the initial relapse manife-
station in a previously treated AML. The occurrence of
MS is quite rare. The male:female ratio is 1.2:1 and the
median age is 56 years (range 1 month - 89 years) [1].
The association between tuberculosis and hematolo-
gical neoplasms has been recognized for many years be-
cause of the T-cell immunodeficiency caused by the un-
derlying disease and/or its treatment. However, the diag-
nosis of tuberculosis may be problematic because the
immunodeficiency may attenuate or mitigate its symp-
toms and also symptoms and signs can be overlapped by
those from the hematologic malignancy [2].
The reported prevalence of tuberculosis in patients
with hematologic malignancies is between 0.72% and
2.6% [2]. Conversely, the diagnosis of a simultaneous
occurrence of a rare hematological neoplasm such as MS
and bone tuberculosis at in itial presentation and from the
same involved site seemed quite unusual and due to its
rarity and interest, we aimed to report this case [3-9].
2. Case Report
A 21-year-old female patient presented cough, sore
throat and a suppurative swollen gum for 10 days prior to
Copyright © 2013 SciRes. JCT
Simultaneous Diagnosis of Myeloid Sarcoma of the Jaw and Mycobacterium tuberculosis Infection
1050
hospital admission. Physical examination revealed mod-
erate pallor and swollen inferior gum. Past medical his-
tory included weight loss, fatigue, hypermenorrhea, pre-
vious use of iron salt for anemia and this subject denied
previous expo sure to chemical agents or pesticides. CBC
revealed Hb 6.5 g/dL, hematocrit 18.4% MCV 97 fL
MCH 34 pg, WBC 18.5 109/µL (1 My/3 Bt/69 Sg/ 1
Eo/0 Ba/20 Ly/6 Mo), Platelets 43 109/µL. The
peripheral blood smear presented with 3% blast cells
(type 1) and granulocytic dysplasia (hypogranular cells
and abnormal nuc lei condensation). Bone marrow aspira-
tion, although hemodiluted, was normocellular, G:E ratio
2.4:1 and there were 6.3% type 1 myeloid blast cells (no
Auer rods). Immunophenotyping from bone marrow re-
vealed 10% blast cells positive for CD34, HLA-DR,
CD-117, CD65dim, CD64dim and CD7. Bone marrow bi-
mopsy showed 100% cellularity, 50% of cells were from
granulocytic precursors, there was evident dysplastic
megakaryocytes and fibrosis grade 2. Immuhistochemis-
try studies were positive for lysozyme, MPO in granulo-
ncytic cells and LCA and CD34 in immature cells. Bone
marrow karyotype was unsuccessful due to absence of
metaphases.
Imaging studies revealed lytic jaw lesions not other
bone was involved (CT scans). Abdominal and thorax
CT scans were unremarkable. Bone scan showed increas-
ed uptake in jaw. Gum biopsy showed increased num-
ber of immature cells positive for LCA and MPO favor-
ing the diagnosis of granulocytic sarcoma.
Given the clinical picture and histological findings
thee diagnosis of AML was established: granulocytic sar-
coma with massive gum infiltration (immature granulo-
cytic cells) and 10% of blasts in bone marrow. The pa-
tient was assigned to receive intent of treatment therapy:
induction chemotherapy (3 + 7 cytarabine 100 mg/m2
D1-D7 and daunorub icin 90mg/m2 D1-D3), an d gum tis-
sue culture was positiv e for Mycobacterium tuberculosis.
No other pathogens were found in tissue culture. Simul-
taneously, a qRT-PCR test confirmed the same bacteria
in the gum tissue. Within two concomitant diagnosis tu-
berculosis and myeloid sarcoma in the same topography
the gum biopsy and bone marrow slides were reviewed
by hematology and pathology team both attempts in or-
der to look up and review for any evidence of tuberculo-
sis granuloma and granuloma-associated necrosis but
neither slides and tissue samples did not demonstrate any
of latter. Since jaw tuberculosis was established she was
treated with isoniazid, rifampicin, pyrazinamide and eth-
ambutol ciprofloxacin and amikacin). Curiously, when
tuberculosis reports were available patient was in nadir
after induction. We were concerned that patient could
develop milliary or disseminated tuberculosis but did not
occur.
Complete remission was achieved and the patient was
submitted for consolidation/intensification (HiDAC x3)
schema and referred to allogeneic HSCT. After induction
and full hematological recovery there was no further
evidence or recurrence of fever and lytic lesions. Cur-
rently patient is under CR and ling follow up (48 months)
did not show recurrence of ei t her AML or tu berc ul osi s.
3. Discussion
According to the 4th edition of the WHO classification
(2008), myeloid sarcoma is categorized into AML and
related precursor neoplasms as a distinct pathologic en-
tity [1]. Differential histological diagnosis at conven-
tional light microscopy are lymphoblastic lymphoma,
Burkitt’s or diffuse large B-cell lymphoma or tissue in-
filtration by a non-hematopoietic tumor. As this implies a
wrong treatment, the application of immunophenotyping,
immunohistochemistry is of paramount importance for
the lineage definition, differential diagnosis and specific
treatment.
This case presented several medical challenges. De-
spite the certainty of current MS in the jaw, bone marrow
was not massively infiltrated by abnormal blast cells. In
the present case dysplastic features were evident in bone
marrow and there were no other medical conditions that
could be related to such morphological abnormalities.
Moreover, MS usually occurs in patients with AML,
MDS, or MPN, but it may present as de novo or rarely
precede peripheral blood or bone marrow involvement,
posing a diagnostic challenge. Plus, the gingival/gum/jaw
anatomical site of MS was quite unusual and in addition
patient presented suppurative gum lesion on the same
anatomical site of the MS. This suppurative gum lesion
which was initially managed with large spectrum anti-
biotics and was found to be caused by tuberculosis. After
induction chemotherapy and proper tuberculostatic treat-
ment there were no clinical or radiological recurrence of
suppurative gum or other lesions elsewhere nor dissemi-
nated tuberculosis. Quite often patients who present
tuberculosis may suffer from other medical conditions
that are associated with poor health status, immunode-
ficiency, chronic conditions including but not limited to
chronic alcohol exposure, malnutrition, drug abuse or
other acquired immunodeficiency diseases which were
not found in this case. Systematic tuberculosis screening
was made after jaw culture was positive for M. tubercu-
losis and no other site was involved with mycobacterium
infection. Tuberculosis diagnosis was performed during
nadir and medical attention was given to assess and treat
any other additional infectious disease which did not
occur. To date the course of disseminated tuberculosis is
usually fatal in immunocompromised patients and the
current present case patient presented successful bone
marrow recovery, achieved complete remission and did
Copyright © 2013 SciRes. JCT
Simultaneous Diagnosis of Myeloid Sarcoma of the Jaw and Mycobacterium tuberculosis Infection
Copyright © 2013 SciRes. JCT
1051
not present any further infectious disease complications
throughout AML chemotherapy treatment [2-9].
In a previous survey, tuberculosis was detected in
2.6% (n = 24 in 129) in all hematological neoplasms in a
10-year period [1]. In this published series no tuber-
culosis wa s diagnosed in AML or MS only MD S (n = 2) .
The univariate analysis of that study identified several
factors associated with a high risk of tuberculosis: chro-
nic lymphocytic leukemia, malnutrition (OR 38.78, 95%
CI 2.35 - 639, p = 0.05), previous corticosteroid use and
use of fludarabine. In the case presented we could only
envision poor dental care but not any of the conditions
associated with a high risk tuberculosis.
According to a study published on Leukemia (2007)
[1], with 92 MSs so far evaluated there were no signi-
ficant differences in survivor incidence between the group
with de novo tumor and the one with concomitant or
those ones with a previous hematological disorder. In-
terestingly, all survivors achieved complete remission
following the first line of therapy. By con trast, only eight
out of sixty patients who deceased obtained complete
remission following the initial therapeutic approach. Six
out of seven survivors underwent allogeneic bone mar-
row transplant, the remaining one having received seve-
ral courses of conventional chemotherapy. Notably, ac-
cording to that study, the patients who died of disease
within the group of transplanted patients (two following
Autologous HSCT used as initial therapy and two who
received allogeneic HSCT as salvage therapy) experi-
enced prolonged survival (from 8 months to six years;
mean 41 months). By contrast, the mean survival of the
patients who underwent chemotherapy, imatinibmesylate,
surgery and radiotherapy were as follows: 7.1 months,
5.6 months, 36 days and 1 week respectively. The sur-
vival rates at 48 months of the patients who did and did
not undergo auto/allogeneic HSCT are 76% vs 0%; p =
0.0000. The clinical behavior and response to therapy
were not influenced by age, sex, anatomic site(s) involv-
ed, clinical presentation, previous clinical history, his-
tology type, phenotype and cytogenetic findings. These
data support the indication of bone marrow transplant as
a consolidation therapy. We concluded this patient had a
synchronous diagnosis of myeloid sarcoma and jaw tu-
berculosis.
REFERENCES
[1] S. A. Pileri, S. Ascani and M. C. Cox, “Myeloid Sarcoma:
Clinico-Pathologic, Phenotypic and Cytogenetic Analysis
of 92 Adult Patients,” Leukemia, Vol. 21, 2007, pp. 340-
350. doi:10.1038/sj.leu.2404491
[2] F. A. Silva, J. O. Matos, Q. M. F. C. de and M. Nucci,
“Risk Factors for and Attributable Mortality from Tuber-
culosis in Patients with Hematologic Malignances,” Ha-
ematologica, Vol. 90, No. 8, 2005, pp. 1110-1115.
[3] A. Klossek, C. Dannenberg, M. R. Feuerhahn and D.
Körholz, “Pulmonary Tuberculosis in a Child Receiving
Intensive Chemotherapy for Acute Myeloblastic leukemia,”
Journal of Pediatric Hematology/Oncology, Vol. 26, No.
1, 2004, pp. 64-67.
doi:10.1097/00043426-200401000-00019
[4] I. K. Neonakis, M. G. Alexandrakis, Z. Gitti, G. Tsirakis,
E. Krambovitis and D. A. Spandidos, “Miliary Tuberculo-
sis with No Pulmonary Involvement in Myelodysplastic
Syndromes: A Curable, yet Rarely Diagnosed, Disease:
Case Report and Review of the Literature,” Annals of
Clinical Microbiology and Antimicrobials, Vol. 7, 2008,
p. 8. doi:10.1186/1476-0711-7-8
[5] Y. C. Chen, J. M. Sheen, L. T. Huang, K. S. Wu and C. C.
Hsiao, “Disseminated Tuberculous Myositis in a Child
with Acute Myelogenous Leukemia,” Pediatrics & Neo-
natology, Vol. 50, No. 2, 2009, pp. 74-77.
doi:10.1016/S1875-9572(09)60037-1
[6] J. S. Ahn, D. H. Yang, Y. K. Kim, S. H. Cho, I. Y. Kim, J.
J. Lee, I. J. Chung and H. J. Kim, “Multiple Intracranial
Tuberculomas Mimicking Granulocy tic Sarcomas in Acute
Myeloid Leukemia,” Journal of Korean Medical Science,
Vol. 22, 2007, pp. S171-S173.
doi:10.3346/jkms.2007.22.S.S171
[7] P. C. Jutte, R. Van Altena, E. Pra s and C. J. Thijn, “Cause s
of Misdiagnosis and Mistreatment of Spinal Tuberculosis
with Radiotherapy in Nonendemic Areas: A Pitfall in Di-
agnosis and Treatment: Hazards of Radiotherapy on the
Tuberculous Lesion,” Spine, Vol. 30, No. 11, 2005, pp. E
300-E304.
[8] M. Gesse, G. Mall, D. Fritze and M. Gartenschläger,
“Granulocytic Sarcoma of the Tonsils Associated with
Myelodysplastic Syndrome,” Deutsche Medizinische Wo-
chenschrift, Vol. 127, No. 50, 2002, pp. 2673-2676.
[9] S. H. Swerdlow, E. Campo, N. L. Harris, E. S. Jaffe, S. A.
Pileri, H. Stein, J. Thiele and J. W. Vardiman, “WHO
Classification of Tumours of Haematopoietic and Lym-
phoid Tissues,” 2008.