|Year : 2022 | Volume
| Issue : 1 | Page : 32-35
An unusual case of coronavirus disease 2019 associated with chronic myeloid leukemia in a young male presenting with priapism
Banyameen Iqbal, Prtyush Mishra, Rakesh Kumar
Department of Pathology, Dr. D.Y Patil Medical College, Hospital and Research Centre, Pune, Maharashtra, India
|Date of Submission||02-Feb-2021|
|Date of Decision||27-Nov-2021|
|Date of Acceptance||06-Dec-2021|
|Date of Web Publication||02-Sep-2022|
Dr. Banyameen Iqbal
Department of Pathology, Dr. D.Y Patil Medical College, Hospital and Research Centre, Pimpri, Pune - 411 018, Maharashtra
Source of Support: None, Conflict of Interest: None
Patients with chronic myeloid leukemia (CML) usually present in the elderly age and rarely in the adolescents and young adults (AYA) groups. In this case report, we describe a case of a 28-year-old male who presented to the hospital with priapism and pain abdomen along with a positive history of coronavirus disease 2019 (COVID-19) disease. The patient was diagnosed as CML based on his peripheral blood smear and bone marrow aspiration findings. These findings were confirmed by fluorescence in situ hybridization (FISH) which showed the presence of breakpoint cluster region/ABL1 fusion gene. The patient was treated with tyrosine kinase inhibitor along with surgical shunt for priapism which showed clinical improvement. There is a very limited understanding of disease management and survival outcomes in this group of patients; therefore, the aim of this case report is to understand the association of COVID-19 infection in patients of AYA group suffering from CML.
Keywords: Chronic myeloid leukemia, coronavirus disease-19, priapism
|How to cite this article:|
Iqbal B, Mishra P, Kumar R. An unusual case of coronavirus disease 2019 associated with chronic myeloid leukemia in a young male presenting with priapism. J Med Soc 2022;36:32-5
|How to cite this URL:|
Iqbal B, Mishra P, Kumar R. An unusual case of coronavirus disease 2019 associated with chronic myeloid leukemia in a young male presenting with priapism. J Med Soc [serial online] 2022 [cited 2022 Oct 4];36:32-5. Available from: https://www.jmedsoc.org/text.asp?2022/36/1/32/355579
| Introduction|| |
Chronic myeloid leukemia (CML) appears in all age groups, yet the incidence increases with age. In western countries, the median age at the diagnosis is 64 years and around 54 years in other clinical studies. A small percentage of patients diagnosed with CML are children and young adults. CML represents about 3% of newly diagnosed childhood leukemia's. CML is a myeloproliferative neoplasm characterized by the dysregulated production and uncontrolled proliferation of mature and maturing granulocytes with fairly normal differentiation. Commonly known as a disease of the elderly, CML rarely affects the pediatric group (0–14 years), or adolescent and young adults (15–29 years). In India, Dikshit et al. reported that during 2001–2005, age-specific rate of CML per 100,000 populations in paediatric age group was 0.04 and in young adults and adolescent groups was 0.22. Coronavirus disease 2019 (COVID-19) is a respiratory viral disease that commonly presents with mild symptoms. However, it can cause serious complications such as acute respiratory disease, especially in patients with comorbidities. As it is a new disease, the data about the risk in patients with hematological malignancies are limited.
| Case Report|| |
In this report, a 28-year-old male with no prior medical history presented with chief complaints of painful erection of penis for 4 days associated with weight loss of 5–6 kg in last 2 months, lump in the abdomen associated with pain for 2 months, fever on and off for 1 month associated with night sweats, chills, and rigor and bleeding per rectum for 2 months. The patient also had a history of fever on and off for the last 1 month which was associated with night sweats, chills, rigors, and bleeding per rectum for the last 2 months. The patient has tested positive for COVID-19, 2 months back and has received treatment as per defined Indian Council of Medical Research protocol and remained under home quarantine for 21 days. He also has an addiction history of alcohol consumption for 8 years and tobacco chewing for 7 years. On general physical examination, the vitals were stable with positive finding of pallor, bony tenderness and the penis was erect, firm and tender with superficial venous engorgement. The systemic examination revealed the presence of massive splenomegaly and moderate hepatomegaly. His laboratory investigations were pertinent for hemoglobin of 8.6 g/dL, platelets count of 379,000/cmm. His white blood cells count of 344,900/cmm and the peripheral smear revealed a range of granulocytic cells with <10% blasts. 10% neutrophils, 05% bands, 20% metamyelocytes, 20% myelocytes, 10% promyelocytes, 5% blasts, 1% lymphocytes, and 4% eosinophils [Figure 1]. Serum chemistries were unremarkable. Imaging comprised of ultrasonography of the abdomen confirmed extensive splenomegaly (22 cm in size) and hepatomegaly (17 cm in size). Bone marrow aspirate and core biopsy specimen revealed morphologic findings consistent with CML in chronic phase, including marked hypercellularity, myeloid hyperplasia, and prominent granulocytic lineage without evidence of increased blasts. Reticulin stain showed extensive bone marrow fibrosis [Figure 2], [Figure 3], [Figure 4]. Fluorescent in situ hybridization (FISH) was positive for breakpoint cluster region (BCR)/ABL1 fusion genes.
|Figure 1: Photomicrograph of peripheral blood smear showing range of granulocytic cells (Leishman ×400)|
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|Figure 2: Photomicrograph of bone marrow aspiration showing granulocytic precursors (Leishman ×400)|
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|Figure 3: Photomicrograph of bone marrow biopsy showing hypercellular marrow and myeloid hyperplasia (H and E ×400)|
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The patient underwent an emergency procedure of “distal shunt” named the “Winter's Technique” for emergency management of priapism. He was further treated with Imatinib and Hydroxyurea. The patient's clinical status then improved, and he was subsequently discharged from the hospital with a follow-up with the hematology clinic in the outpatient setting.
| Discussion|| |
The case reported here presented with all the features of CML except for the age which belonged to much uncommon adolescents and young adults (AYA) group along with recent history OF COVID-19. CML has always been known as a disease of the elderly, yet some current literature reports that AYA can also have CML but with inadequate knowledge of the disease survival outcomes in this specific age group. CML is preferably distinguished by t (9;22) (q34; q11.2) genetic translocation leading to fusion of Abelson murine leukemia (ABL1) gene with BCR gene that results in ungovernable activity of the tyrosine kinase BCR-ABL1 fusion protein, which makes this genetic translocation a target for novel tyrosine kinase inhibitor therapy. The course of this disease results in the deregulation of hematopoiesis, resulting in a proliferation of immature granulocytes. Massive splenomegaly is one of the most prevalent findings during the physical examination in almost 50% of these patients. These patients present with anemia resulting in fatigue and can also show weight loss and abdominal pain because of splenomegaly. In our patient along with all the above-mentioned features, priapism was the main presentation which is considered to be a complication of hyperviscosity due to leukocytosis and venous obstruction as a result of thrombi and microthrombi. Priapism is a rare condition itself with an incidence of 1.5 cases per 100,000 person-in years. Hematological conditions cause 20% of cases and priapism only secondary to penile metastasis which being rare yet one of its etiological factors. Priapism is seen in between 1% and 5% of male patients with all types of leukemia. However, as a presenting feature of CML, priapism is rare in male patients, occurring only in 1%–2%.
AYA group population often presents differently compared with other age population, more often with a much higher WBC count and higher incidence of a larger spleen size in comparison to other age groups. CML having a triphasic nature, the chronic phase being the most common phase reported to be present in up to 90% of cases. The chronic phase has a potential to progress into accelerated and blastic phases. Therefore, it exhibits a high variability in presentation and prognosis.
Severe acute respiratory syndrome coronavirus 2 (SARS-CoV 2) is a zoonotic beta virus like Middle East Respiratory Syndrome and SARS-COV 1, which causes respiratory illness called COVID-19 or coronavirus disease. Most of cases are present with simple symptoms such as fever, cough, and fatigue but few cases are severe and might require mechanical ventilation. Although the disease has severe complications when associated with comorbidities, yet there is no evidence of the disease severity in hematological malignancies such as CML, AML, or ALL which might place the patient at high risk. Lymphopenia is the most common laboratory finding in COVID-19; however, leukocytosis and leukopenia have been reported as well. Thrombocytopenia is more common in patients with critical diseases. Some patients also develop cytokine storm or secondary hemophagocytic lympho-histiocytosis, which is characterized by cytopenia and other features.
| Conclusion|| |
Further study should be undertaken to find out the true incidence of CML in pediatric and young adolescent age groups along with its correlation in COVID-19. Furthermore, the importance of prompt diagnosis and management of priapism cannot be undermined, as there is definite incidence of impotence following this condition.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]