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Chronic Myeloid Leukemia: Chronic Phase Presenting as Extramedullary

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Article  in  Cureus · December 2022

DOI: 10.7759/cureus.32691

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5 authors, including:

Vedant Avinash Nanote Ojas Mahajan

Kazan State Medical University Datta Meghe Institute of Medical Sciences (Deemed University ), India, Wardha
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Shilpa Bawankule Lalit Raut

Dattameghe institute of medical sciences india Vancouver General Hospital
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Open Access Case
Report DOI: 10.7759/cureus.

Chronic Myeloid Leukemia - Chronic Phase

Presenting as Extramedullary Presentation
Vedant A. Nanote 1 , Ojas A. Mahajan 2 , Shilpa A. Gaidhane 2 , Swapnil D. Parve 3, 4, 5, 6 , Lalit Raut 2

1. Dept. of Primary Care & General Practice, Kazan State Medical University, Wardha, IND 2. Department of Medicine,
Jawaharlal Nehru Medical College, Wardha; Datta Meghe Institute of Medical Sciences, Wardha, IND 3. Dept. of Primary
Care & General Practice, Kazan State Medical University, Kazan, RUS 4. Dept. of Medicine, University of Vermont
Robert Larner, MD School of Medicine, Burlington, USA 5. Global Health Program, Nuvance Health, Danbury, USA 6.
Research and Development, Datta Meghe Institute of Medical Sciences (DU), Sawangi Meghe, IND

Corresponding author: Shilpa A. Gaidhane, drshilpagaidhane@gmail.com

Abstract
Chronic myeloid leukemia (CML) is a myeloproliferative disorder characterized by immature granulocytes in
the peripheral blood and bone marrow. In 95% of cases, it is always associated with the presence of the
Philadelphia chromosome, which is characterized by the presence of reciprocal translocation between
chromosome 9 and 22. However, 7% -17% of patients also have an extramedullary proliferation of skin,
lymph nodes, bone, or central nervous system (CNS), which could be either myeloid, lymphocytic or mixed
lineage in origin. An extramedullary myelogenous neoplasm termed myeloid sarcoma (MS) can originate
from myeloblasts or immature myeloid cells. Due to the green, gross appearance caused by the
myeloperoxidase enzyme in immature myeloid cells, it is also known as chloroma. According to WHO
guidelines, it is a tumor composed of myeloid blasts, mature or immature. Here we report an old female
patient with chronic myeloid leukemia - chronic phase came for imatinib therapy and presented as myeloid
sarcoma in the right parotid gland.

Categories: Internal Medicine, Hematology

Keywords: tyrosine kinase inhibitors (tkis) therapy, parotid gland, chronic phase, chronic myeloid leukemia, myeloid
sarcoma

Introduction
A patient undergoing Tyrosine Kinase Inhibitors (TKIs) therapy for chronic myeloid leukemia (CML) -
chronic phase presented as myeloid sarcoma in the right parotid gland. Myeloid sarcoma is an
extramedullary tumor composed of immature myeloid cells that destroy the underlying tissue
architecture. It is usually associated with acute myelogenous leukemia but can be associated with
myeloproliferative neoplasms, myelodysplastic disorders, or myeloproliferative/myelodysplastic syndromes.
[1] If undetected it may delay appropriate chemotherapy and jeopardize survival. [2,3] Myeloid sarcoma (MS)
is a tumor mass which arises in anatomical sites other than the bone marrow. [4] Myeloid sarcoma mostly
occurs during the accelerated phase or the blast phase in the bone marrow and peripheral blood but rarely in
chronic phase. However, we present a patient presenting with chronic myeloid leukemia - chronic phase
having myeloid sarcoma in the right parotid gland.

Case Presentation
Patient Information:
A 69-year-old female patient came to the outpatient department of Medicine for imatinib therapy and
presented with complaints of dyspnea on exertion, lassitude and swelling over the right jaw and right
parotid gland (Figure 1). A month ago, the patient seemed to be doing well when she observed a mass around
her right parotid gland which was insidious in onset, initially not present and gradually progressed to its
present size. Physical examination revealed an enlarged cervical lymph node, hepatosplenomegaly, and
parotid swelling. The patient is known hypertensive since 10 years on regular medications. In 2019, she
visited an Internal Medicine outpatient clinic for the first time due to swelling over her legs due to trauma.
Bone marrow biopsy and peripheral smear were done which showed a '’Myeloid Bulge’’ and was suggestive
of chronic myeloid leukemia-chronic phase. The patient has a history of chronic myeloid leukemia-chronic
phase from past 3 years on regular medications.

How to cite this article

 FIGURE 1: Patient presenting with Chronic Myeloid Leukemia - Chronic
phase having mass around the right parotid gland

2019 Laboratory Findings:

She was diagnosed to have Chronic Myeloid Leukemia as per the following laboratory investigations
suggestive of peripheral smear showing Normocytic erythrocytes cells with adequate platelet on smear,
basophilia and leukocytosis with TLC of 2,00,000/cu mm (Normal range- 4,000-11,000/cu mm). According to
the criteria for chronic myeloid leukemia - chronic phase, blasts should be less than 10%. Bone marrow
biopsy was suggestive of increased myeloid cells, erythroid cells with normoblastic erythropoiesis, few
megakaryocytes with normal morphology.

Current clinical findings:

Physical examination revealed cervical lymph node enlargement and hepatomegaly. On palpation of the
parotid gland, it revealed a 2x2 cm round, hard, painless, non-tender mass that was soldered to the parotid
gland without skin and subcutaneous infiltration.

Cells Results Normal Range

Myeloblast 04% 0-1.5%

Promyelocytes 08% 2.0-4.1%

Myelocytes 21% 8.2-16%

Metamyelocytes 19% 9.6-24.6%

Band forms 8% 9.5-24.6%

Basophils 4% 0-1%

Polymorphs 28% 40-65%

Lymphocytes 06% 30-50%

TABLE 1: Differential leukocytes count of the patient in 2019

Diagnostic assessment: Current laboratory analysis

Fine needle aspiration cytology (FNAC) was done for mass over the right parotid gland
which revealed myeloperoxidase-positive cells, found in <10% of cells of myeloid lineage in peripheral
smear (Figure 2) and showed infiltration of CML and no parotid tumor was noticed. Ultrasound local site
revealed a well-designed hypoechoic lesion measuring approximately 18 x 16 mm in the superficial lobe of
the right parotid gland. Laboratory investigations were suggestive of hemoglobin - 9gm/dl and total
leukocytes count 83,800 cells/cu mm. RBC mass appeared reduced on peripheral smear. RBC precursors
(intermediate and late normoblasts) are seen. Normocytic normochromic anemia with few microcytes.
Platelets (2.13cu/mm) are adequate on smear. Peripheral smear findings were suggestive of normocytic
anemia, leukocytosis with '' Myeloid Bulge'' with band forms, neutrophils and other immature cell (Figure 3)

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 FIGURE 2: Fine needle aspiration cytology (FNAC) of mass over the
right parotid gland showing myeloperoxidase positive cells

Cells Results Normal Range

Myeloblasts 05% 0-1.5%

Promyelocytes 10% 2.0-4.1%

Myelocytes 12% 8.2-16%

Metamyelocytes 12% 9.6-24.6%

Band forms 04% 9.5-15.3%

Lymphocytes 08% 30-50%

Monocytes 0% 2-8%

Eosinophils 03% 1-4%

Basophils 10% 0-1%

TABLE 2: Current differential leukocytes count

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 FIGURE 3: Peripheral smear of blood sample showing Myeloid Bulge

Therapeutic interventions :
Considering the patient's hypertensive comorbidity we prescribed her combination of Tab Atenolol and
amlodipine (50/5) mg once a day and Tab Ecosprin 75 mg once a day and for Chronic Myeloid Leukemia Tab
Imatinib 400 mg twice daily. The standard treatment for chronic phase CML is tyrosine kinase inhibitor
Imatinib.

Discussion
Our patient has a rare condition in the chronic phase of CML with extramedullary localization of
myelogenous sarcoma. However, most CML patients present with extramedullary symptoms during blast
crises. Here, our patient has clinical laboratory results in the chronic phase but shows extramedullary
parotid swelling and the Fine Needle Aspiration Cytology (FNAC) report shows myeloperoxidase positive
cells, indicating myelosarcoma. The patient lives in a rural Indian village, uneducated and unaware of her
illness. She is not very financially stable and cannot afford the cost of her treatment. [1] Economically she is
not much stable hence she cannot afford the cost of treatment. Therefore, hospitals provide their treatment
with funding under government programs. After initial treatment in the hospital, the patient improved and
was discharged. As a mentioned earlier patient is economically not stable and hence, cannot opt for
advanced treatment options. [2] As of 2019, patient is still on Imatinib therapy, no changes can be seen in
her laboratory analysis parameters, so drug monitoring should be done to rule out imatinib resistance in a
patient, and second-line tyrosine kinase inhibitors should be prescribed such as dasatinib, bosutinib,
nilotinib or third line: ponatinib. We can also try dose escalation but in a patient, we can see the suboptimal
response to imatinib therapy. Disease monitoring by complete blood counts done once in every two weeks
[3,4]. Bone marrow biopsies have to be done in the period of every 6 months to check the cytogenic
responses. Once a bone marrow biopsy and cytogenic response is achieved quantitative RT-PCR(qPCR) for
BCR-ABL1 should be done on peripheral blood at 3-6 month intervals. [5] If after monitoring the disease, the
patient is diagnosed under accelerated phase or blast crisis it should be treated accordingly. Accelerated
phase is treated with high doses of cytarabine (HDAC) and hydroxyurea (Hydrea). Blasts crisis is treated like
acute myeloid leukemia - First step is induction chemotherapy, we may follow regimes such as DA 3+10 -
Daunorubicin 50mg/m2 on days 1,3,5 and cytarabine 100mg/m2 twice a day on days 1-10. DA 3+8 -

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 Daunorubicin 50mg/m2 day 1,3,5 and cytarabine 100mg/m2 twice a day on days 1-8. Second step is
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consolidation chemotherapy we may follow regimes such as MACE - Etoposide 100mg/m2 on days 1-5,
Amsacrine 100mg/m2 on days 1-5, Cytarabine 200mg/m2 on days1-5. [6]

Conclusions
This is a unique case as there is extramedullary presentation i.e. Myeloid Sarcoma in Parotid Gland in the
CML-in chronic phase. Managing this situation requires timely monitoring, optimal dose of drugs and good
adherence, which is challenging for the developing rural population of India due to financial and economic
challenges.

Additional Information
Disclosures
Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In
compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services
info: All authors have declared that no financial support was received from any organization for the
submitted work. Financial relationships: All authors have declared that they have no financial
relationships at present or within the previous three years with any organizations that might have an
interest in the submitted work. Other relationships: All authors have declared that there are no other
relationships or activities that could appear to have influenced the submitted work.

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