Authors

  • Ortik Almamatov
    Samarkand Regional Multidisciplinary Medical Center
  • Akbar Fayziyev
    Samarkand Regional Multidisciplinary Medical Center
  • Oyjamol Uzakova
    Samarkand Regional Multidisciplinary Medical Center

DOI:

https://doi.org/10.71337/inlibrary.uz.ijms.114377

Abstract

Acute promyelocytic leukaemia (APL) is a distinct subtype of acute myeloid leukaemia characterized by a specific genetic translocation and a high risk of coagulopathy. The coexistence of APL with renal pathology complicates both diagnosis and treatment, requiring a tailored multidisciplinary approach. We report a case of a 47-year-old male with APL complicated by acute kidney injury, presenting with bleeding, pancytopenia, and disseminated intravascular coagulation. Diagnosis was confirmed by bone marrow examination and molecular testing for the PML-RARA fusion gene. Treatment included all-trans retinoic acid and reduced-dose arsenic trioxide, adjusted for renal impairment, along with supportive care addressing coagulopathy, tumour lysis syndrome prophylaxis, and infection management. The patient achieved complete hematologic and molecular remission with restoration of renal function. This case underscores the importance of individualized therapy and vigilant monitoring in managing APL patients with renal complications, demonstrating that successful outcomes are achievable despite significant comorbidities.

 

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A CLINICAL CASE OF ACUTE PROMYELOCYTIC LEUKAEMIA IN A PATIENT

WITH RENAL PATHOLOGY

Almamatov Ortik Ibragimovich,

Samarkand Regional Multidisciplinary Medical Center – Department of Therapy, Therapist.

Fayziyev Akbar Ubaydullo ugli,

Samarkand Regional Multidisciplinary Medical Center – Department of Therapy, Therapist.

Uzakova Oyjamol Narzullaevna

,

Samarkand State Medical University, Department of Hematology, Trainee-Assistant.

Abstract.

Acute promyelocytic leukaemia (APL) is a distinct subtype of acute myeloid

leukaemia characterized by a specific genetic translocation and a high risk of coagulopathy.

The coexistence of APL with renal pathology complicates both diagnosis and treatment,

requiring a tailored multidisciplinary approach. We report a case of a 47-year-old male with

APL complicated by acute kidney injury, presenting with bleeding, pancytopenia, and

disseminated intravascular coagulation. Diagnosis was confirmed by bone marrow

examination and molecular testing for the PML-RARA fusion gene. Treatment included all-

trans retinoic acid and reduced-dose arsenic trioxide, adjusted for renal impairment, along

with supportive care addressing coagulopathy, tumour lysis syndrome prophylaxis, and

infection management. The patient achieved complete hematologic and molecular remission

with restoration of renal function. This case underscores the importance of individualized

therapy and vigilant monitoring in managing APL patients with renal complications,

demonstrating that successful outcomes are achievable despite significant comorbidities.

Key words:

Acute promyelocytic leukaemia; Renal pathology; Acute kidney injury; All-

trans retinoic acid; Arsenic trioxide; Disseminated intravascular coagulation; Molecular

remission; Multidisciplinary management

Introduction

Acute promyelocytic leukaemia (APL) is a distinct and highly aggressive subtype of acute

myeloid leukaemia (AML), characterised by the accumulation of abnormal promyelocytes in

the bone marrow and peripheral blood. APL is defined cytogenetically by the

t(15;17)(q24;q21) translocation, which results in the PML-RARA fusion gene. This

molecular hallmark not only underpins the disease's pathogenesis but also provides a unique

opportunity for targeted therapy, which has significantly improved survival rates in recent

decades. The introduction of all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) has

transformed APL from one of the most fatal forms of leukaemia into one of the most curable

haematological malignancies.

However, the clinical course of APL can be complicated by severe coagulopathy, early

haemorrhagic events, and organ dysfunction, all of which may be life-threatening if not

promptly recognised and managed. Renal pathology—whether pre-existing or secondary to

the disease or its treatment—adds further complexity to diagnosis and therapeutic planning.

Acute kidney injury (AKI) in patients with APL may result from tumour lysis syndrome,


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sepsis, nephrotoxic drug exposure, or direct leukemic infiltration, significantly impacting

treatment tolerability and overall prognosis.

The management of APL in patients with renal impairment presents a particular challenge,

as the pharmacokinetics and toxicity profiles of ATRA, ATO, and supportive therapies may

be altered. Dose adjustments, careful fluid and electrolyte management, and

multidisciplinary coordination are essential to achieve remission without causing further

renal compromise. Moreover, renal dysfunction can mask early clinical signs of leukaemia,

delay diagnosis, or confound laboratory assessments such as creatinine-based renal function

estimation and drug clearance.

This case report presents the clinical course of a patient diagnosed with acute promyelocytic

leukaemia in the setting of underlying renal pathology. It highlights the diagnostic

challenges, therapeutic modifications, and outcomes observed, and discusses the

implications for clinical practice. Through this case, we aim to contribute to the growing

div of evidence regarding the management of haematological malignancies in patients with

multi-organ comorbidities, particularly renal disease.

Methods

This clinical observation was conducted at the Samarkand Regional Multidisciplinary

Medical Center in early 2025, with informed consent obtained from the patient and approval

granted by the institutional ethics committee. A comprehensive diagnostic and treatment

protocol was applied, integrating haematological and nephrological approaches. Upon

presentation, the patient underwent a complete blood count, peripheral smear analysis,

coagulation tests, and biochemical profiling to assess kidney function, electrolyte balance,

and the severity of cytopenia. Bone marrow aspiration and biopsy were performed, revealing

a predominance of abnormal promyelocytes, while molecular confirmation of the PML-

RARA fusion gene was established through RT-PCR and FISH testing. This enabled a

prompt diagnosis of acute promyelocytic leukaemia. Given the patient's compromised renal

function at baseline, as indicated by elevated creatinine levels and reduced eGFR, careful

adaptation of the therapeutic regimen was necessary. All-trans retinoic acid (ATRA) was

initiated at standard dosing (45 mg/m²/day), while arsenic trioxide (ATO) was introduced

cautiously at 0.10 mg/kg/day with dose modifications based on daily renal monitoring.

Supportive therapy included prophylaxis for tumour lysis syndrome, transfusions of platelets

and fresh frozen plasma to manage coagulopathy, and empiric broad-spectrum antibiotics

during neutropenic episodes. Fluid management and nephrotoxic drug avoidance were

prioritized to prevent further renal deterioration. Treatment was guided by a

multidisciplinary team consisting of haematologists, nephrologists, and intensive care

specialists, who jointly monitored therapy tolerance, complications such as QT interval

prolongation, and infection risk. Continuous documentation of laboratory values, patient

responses, and treatment adjustments provided the clinical framework for analysing this case

and evaluating the outcomes of personalised treatment in a patient with dual haematologic

and renal pathology.

Results


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The patient, a 47-year-old male, presented with spontaneous mucosal bleeding, fatigue, and

oliguria. Laboratory evaluation revealed pancytopenia: haemoglobin 76 g/L, leukocytes

2.1×10⁹/L, and platelets 18×10⁹/L. The peripheral blood smear showed 65% abnormal

promyelocytes with frequent Auer rods. Coagulation parameters were markedly abnormal—

prothrombin time prolonged, fibrinogen 0.7 g/L, and elevated D-dimer—suggesting

disseminated intravascular coagulation (DIC). Renal assessment showed serum creatinine at

280 µmol/L, blood urea nitrogen of 14 mmol/L, and an estimated glomerular filtration rate

(eGFR) of 28 mL/min/1.73 m², confirming acute renal dysfunction.

Bone marrow aspiration confirmed hypercellularity with >80% promyelocytes, and

cytogenetic analysis identified the t(15;17)(q24;q21) translocation. RT-PCR confirmed the

PML-RARA fusion transcript. Based on these findings, a diagnosis of acute promyelocytic

leukaemia was made in the setting of acute kidney injury (AKI).

ATRA therapy was initiated promptly, and ATO was introduced at a reduced dose due to

renal impairment. The patient was also given prophylactic allopurinol and aggressive

intravenous hydration to prevent tumour lysis syndrome, with careful fluid balance due to

impaired renal excretory function. Platelet and plasma transfusions were administered to

manage ongoing bleeding and correct coagulopathy. On day 4 of treatment, the patient

developed febrile neutropenia, for which broad-spectrum antibiotics were initiated. No

bacterial growth was found in cultures, and fever resolved within 72 hours.

Renal function gradually improved with supportive care; creatinine decreased to 170 µmol/L

by day 14 and normalized (98 µmol/L) by day 25. Haematological response to therapy was

favourable. By day 28, the patient achieved complete remission: bone marrow showed less

than 5% blasts, platelet counts normalized, and peripheral promyelocytes were no longer

detectable. Molecular testing on day 35 showed no detectable PML-RARA transcripts.

Throughout induction therapy, no severe differentiation syndrome or QT prolongation

occurred. The patient tolerated the treatment well after initial adjustments and was

discharged on day 38 with a plan to continue consolidation therapy under close

nephrological monitoring. The outcome demonstrated successful remission of APL

alongside full renal function recovery, indicating the effectiveness of an individualized,

multidisciplinary management approach.

Discussion

Acute promyelocytic leukemia (APL) is a unique subtype of acute myeloid leukemia

characterized by a specific chromosomal translocation t(15;17), resulting in the PML-RARA

fusion gene. This genetic hallmark not only facilitates a targeted therapeutic approach but

also underscores the importance of rapid diagnosis and initiation of treatment due to the

disease’s aggressive clinical course. The coexistence of APL with renal pathology, as

presented in this case, introduces significant challenges in both diagnosis and management,

necessitating careful consideration of therapeutic strategies and supportive care to optimize

outcomes.

The clinical presentation of APL often involves bleeding diatheses resulting from

disseminated intravascular coagulation (DIC), a critical complication observed in up to 80%


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of cases. In our patient, profound thrombocytopenia and altered coagulation parameters

signaled the presence of severe coagulopathy, complicating the clinical picture. This is

consistent with the pathophysiology of APL, where the release of procoagulant substances

by abnormal promyelocytes triggers systemic activation of the coagulation cascade, leading

to fibrin deposition and consumption of platelets and clotting factors. Prompt recognition

and correction of coagulopathy are vital to reducing morbidity and mortality, as

haemorrhagic events remain a leading cause of early death in APL patients.

Renal dysfunction in APL patients may be multifactorial. It can arise as a consequence of

tumor lysis syndrome, nephrotoxic effects of chemotherapy, infection-related sepsis, or

direct leukemic infiltration. In this case, the patient’s acute kidney injury (AKI) presented a

significant obstacle to standard treatment protocols, necessitating modifications in dosing

and close monitoring to avoid further nephrotoxicity. The reduced dose of arsenic trioxide

(ATO) was critical in minimizing renal adverse effects while maintaining therapeutic

efficacy. This cautious approach highlights the need for individualized treatment regimens

based on comorbid conditions.

The use of all-trans retinoic acid (ATRA) remains a cornerstone in APL therapy, promoting

differentiation of malignant promyelocytes into mature granulocytes. Importantly, ATRA is

generally well tolerated in patients with renal impairment, making it suitable for initial

induction therapy in such complex cases. In contrast, arsenic trioxide, although highly

effective in achieving remission, carries a risk of QT interval prolongation and electrolyte

disturbances, which can be exacerbated by compromised renal clearance. In this patient,

daily renal function monitoring and electrocardiographic surveillance facilitated timely dose

adjustments and prevented severe cardiac toxicity.

Management of tumor lysis syndrome (TLS) is another critical aspect, particularly in

patients with pre-existing renal pathology. Aggressive hydration, uric acid reduction with

allopurinol, and close electrolyte monitoring were integral components of care to prevent

exacerbation of renal injury. The favorable renal recovery observed post-treatment

underscores the effectiveness of these preventive measures.

Infectious complications represent a common challenge during APL treatment due to

profound neutropenia and immunosuppression. The occurrence of febrile neutropenia in this

patient was managed successfully with empiric broad-spectrum antibiotics and careful

supportive care, without progression to sepsis. This outcome reflects the importance of early

recognition and prompt intervention in preventing life-threatening infections.

This case also emphasizes the role of multidisciplinary collaboration in managing patients

with complex comorbidities. The involvement of haematologists, nephrologists, intensivists,

and pharmacists enabled a comprehensive approach that balanced effective leukaemia

treatment with renal protection and supportive management. Such coordinated care is

essential in tailoring therapy, monitoring adverse effects, and improving overall patient

prognosis.

Despite the challenges posed by renal impairment, the patient achieved complete

hematologic and molecular remission, illustrating that with appropriate dose adjustments

and supportive strategies, standard APL treatment protocols can be successfully


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implemented. The absence of severe differentiation syndrome, a potentially fatal

complication of ATRA therapy, further contributed to the positive outcome.

Conclusion

In conclusion, this case illustrates the complexity of treating acute promyelocytic leukemia

in the presence of renal dysfunction. It underscores the necessity for early diagnosis,

individualized therapy, vigilant monitoring, and multidisciplinary management to optimize

treatment efficacy and minimize toxicity. Future studies with larger patient cohorts are

needed to establish standardized protocols for managing APL complicated by renal

pathology and to explore novel therapeutic agents with improved safety profiles in this

vulnerable population.

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Абдураҳмонов, Б. М., & Усмонов, А. Х. (2020). Клинические особенности и лечение острого промиелоцитарного лейкоза. Вестник гематологии, 12(3), 45-53.

Ахмадов, Р. Т., & Каримова, М. С. (2019). Особенности течения лейкемий у пациентов с почечной патологией. Ташкентский медицинский журнал, 7(2), 112-118.

Döhner, H., Estey, E., Grimwade, D., Amadori, S., Appelbaum, F. R., Büchner, T., ... & Bloomfield, C. D. (2017). Diagnosis and management of acute myeloid leukemia in adults: 2017 ELN recommendations from an international expert panel. Blood, 129(4), 424-447. https://doi.org/10.1182/blood-2016-08-733196

Иванова, Н. В., & Петрова, Е. А. (2018). Роль коагулопатий при остром промиелоцитарном лейкозе. Российский журнал гематологии, 14(1), 27-35.

Махмудов, И. Ш., & Юсупова, Л. Б. (2021). Иммунная тромбоцитопения и почечная недостаточность: клинические аспекты. Узбекский медицинский журнал, 3(4), 59-65.

Sanz, M. A., & Fenaux, P. (2019). Management of acute promyelocytic leukemia: recommendations from an expert panel on behalf of the European LeukemiaNet. Blood, 133(15), 1630-1643. https://doi.org/10.1182/blood-2018-11-881040

Shaikh, S., & Hoda, D. (2019). Acute promyelocytic leukemia and renal complications: clinical challenges and treatment strategies. Leukemia Research, 85, 106205. https://doi.org/10.1016/j.leukres.2019.106205

Тураева, Г. Р., & Абдуллаева, Д. Х. (2022). Особенности лечения лейкемии у больных с почечной патологией. Журнал клинической медицины, 5(2), 99-105.

Умаров, З. М., & Нурматов, А. Р. (2020). Современные подходы к диагностике и лечению острых лейкемий. Самаркандский медицинский вестник, 1(1), 15-22.

World Health Organization. (2016). Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition). Lyon, France: IARC.

Абдиев, К., Махмонов, Л., Мадашева, A., & Маматкулова, Ф. (2021). Business games in teaching hematology. Общество и инновации, 2(6), 208-214.

Махмудова, А. Д., Жураева, Н. Т., & Мадашева, А. Г. (2022). НАСЛЕДСТВЕННЫЙ ДЕФИЦИТ ФАКТОРА СВЕРТЫВАНИЯ КРОВИ Х-БОЛЕЗНЬ СТЮАРТА-ПРАУЭРА. Биология, 4, 137.

Madasheva, A. G., Yusupova, D. M., & Abdullaeva, A. A. EARLY DIAGNOSIS OF HEMOPHILIA A IN A FAMILY POLYCLINIC AND THE ORGANIZATION OF MEDICAL CARE. УЧЕНЫЙ XXI ВЕКА, 37.

Мадашева, А. Г. (2022). Клинико-неврологические изменения у больных гемофилией с мышечными патологиями. Science and Education, 3(12), 175-181