Hip joint arthroplasty was successfully performed for patient with clonal cytopenia of undetermined significance: a case report

Introduction

Wimazal et al. had reported idiopathic cytopenia of undetermined significance (ICUS), described as a disease of bone marrow hematopoietic failure that was not yet clearly diagnosed since 2007 (1). It found that about 35% of ICUS patients would experience clonal mutations with the development of second-generation gene sequencing technology (2), and defined ICUS patients with clonal mutations as clonal cytopenia of undetermined significance (CCUS) (2,3). It was a disease characterized by a decrease in one or more hematopoietic systems, but it couldn’t be explained by any other blood or non-blood related diseases (4). The abnormality of the hematopoietic system seriously hindered the implementation progress of surgical procedures. In addition, preoperative anemia or receiving allogeneic blood transfusion can increase perioperative risk, increase postoperative infection rate, prolong hospitalization time, and increase mortality rate.

Avascular necrosis of the femoral head (ANFH) is a common orthopedic disease in clinical practice. At present, total hip arthroplasty (THA) has become the main therapy method for late stage osteonecrosis of femoral head (5). CCUS complicated with ANFH is relatively rare in clinical practice, and CCUS patients who require joint replacement surgery face significant surgical risks. How to help such patients smoothly get through the perioperative period and achieve good functional rehabilitation is the problem we are facing. At present, joint replacement surgery for CCUS patients has not been reported in international medical journals. We report one case suffering from CCUS and undergoing THA admitted to the Department of Orthopedic, Luoyang Orthopedic-Traumatological Hospital of Henan Province (Henan Provincial Orthopedic Hospital) and review the case data reports in previous literature to enhance clinical physicians’ systematic understanding of this type of disease. We present this article in accordance with the CARE reporting checklist (available at https://acr.amegroups.com/article/view/10.21037/acr-24-279/rc).

Case presentation

The patient’s diagnosis and treatment process can be seen in Figure 1.

Figure 1 Patient diagnosis and treatment flowchart. ANFH, avascular necrosis of the femoral head; CCUS, clonal cytopenia of undetermined significance; THA, total hip arthroplasty.

A male patient, 35 years old, Buddhist monks, due to “bilateral hip pain and restricted mobility for 3 years, worsening for 1 month” came to Luoyang Orthopedic-Traumatological Hospital of Henan Province (Henan Provincial Orthopedic Hospital) for treatment on January, 2024. The patient was treated in the hematology department of other hospital for “recurrent low-grade fever for 3 months” in January 2019. The laboratory test results showed a decrease in the number of blood cells in the hematopoietic systems. Bone marrow biopsy indicates low bone marrow proliferation (about 40%), reduced granulocyte to erythroid ratio, and low megakaryocyte count. Immunohistochemical results showed: CD34 (<2%), CD117 (mastocyte+), MPO (granulocyte+), lysozyme (partially+), CD42b (megakaryocyte+), CD61 (megakaryocyte+), CD235a (erythroid+), CD138 (plasma cell+), Kappa (+), Lambda (+). Chromosome karyotype analysis results: 46,XY,del (1)(q11q44),t(1;21)(q11;P11)[3]46,XY[17]; chromosomal structural abnormalities can be seen in the dividing phase. The patient was diagnosed with CCUS after completing the relevant blood system examination in February 2019. The patient was diagnosed with bilateral ANFH in May 2021.

Although medication was administered, there was no significant relief in hip pain symptoms. The X-ray examination of the patient showed severe collapse of both femoral heads, narrowing of the hip joint space, and subluxation of the left femoral head in our hospital (Figure 2). Computed tomography (CT) examination showed that the patient’s bilateral femoral head joint surfaces were fractured, and multiple cystic changes and necrosis occurred within the femoral head (Figure 2). Hip joint magnetic resonance imaging (MRI) examination showed mixed signals in the femoral head, as well as typical “double line sign” and “map sign” manifestations (Figure 2). The patient was diagnosed with bilateral ANFH [Association Research Circulation Osseous classification stage IV (ARCO IV)] according to the diagnostic criteria of the ARCO (6).

Figure 2 Preoperative imaging examination of the patient. (A) Pelvic X-ray examination revealed bilateral femoral head collapse in the patient. (B) Hip CT examination showed bilateral hip joint surface fragmentation and patchy sclerosis in the femoral head of the patient. (C,D) Hip MRI examination showed the presence of “double line sign” and “map sign” necrosis in both femoral heads. CT, computerized tomography; L, left; MRI, magnetic resonance imaging.

The preoperative laboratory test results of the patient showed severe deficiencies in red blood cells, granulocytes, and platelets, elevated venous blood glucose, and no significant abnormalities in chest CT, lung function, and ultrasound examinations of abdominal, cardiac, and lower limbs vascular. We administered multiple component infusions of red blood cells and platelets before surgery. To increase the number of platelets, we orally took 75 mg of eltrombopag tablets daily. We subcutaneously injected recombinant human granulocyte colony-stimulating factor (300 µg) the following day to increase granulocyte levels. We maintained the intravenous glucose of this patient through continuous insulin administration and a strict diabetic diet. We maintained the patient’s blood levels, blood sugar, biochemistry, and coagulation function at a reasonable level during the perioperative period. We performed THA on the patient’s hip joint through the posterior lateral approach under general anesthesia in January 2024 and March 2024, and the two surgeries went successfully without any adverse events. We performed pelvic X-ray examination after surgery and found that the hip joint prosthesis was in good position (Figure 3). The patient had no obvious discomfort symptoms after getting out of bed and moving around. We followed up for 6 months and both hip functions recovered well (Harris Hip Score =90 points).

Figure 3 Postoperative X-ray image of the patient. (A-C) Pelvic X-ray examination in both positive and lateral positions suggested that the position of both hip prostheses was acceptable. L, left; R, right.

The patient used cefuroxime combined with levofloxacin to prevent infection during the perioperative period, and the drainage tube was removed within 48 hours after surgery. We guided the perioperative nutrition of patients to maintain a positive nitrogen balance. We prevented deep vein thrombosis through bilateral lower limb pneumatic pump therapy, encouraged patients to actively engage in ankle pump function exercise and quadriceps isometric contraction exercise to prevent deep vein thrombosis in the lower limbs, and did not use low molecular weight heparin or other anticoagulant drugs.

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration and its subsequent amendments. Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Discussion

Orthopedic surgery was more invasive, and the incidence of perioperative anemia for artificial arthroplasty was 22% (7). Perioperative anemia may increase the risk of postoperative infection, prolong hospital stay, and lead to increased postoperative mortality (7). Zhang et al. also pointed out that anemia increased the risk of poor wound healing, respiratory, renal, and urinary complications in joint replacement patients, and increased the incidence of sepsis and readmission (8). In addition, it has been suggested that preoperative anemia increases the incidence of periprosthesis infection after arthroplasty. When the preoperative hemoglobin (Hb) was less than 100 g/L, the incidence of postoperative infection was the highest, about 4.23%, while when 100≤ Hb ≤120 g/L, the incidence of infection was about 0.84% (9). Foss et al. found that the decline of walking ability in the first 3 days after hip surgery in anemic patients is considered an independent risk factor for early postoperative inability to walk, which affects the patient’s recovery process (10). Therefore, patients undergoing joint replacement surgery should actively correct their anemia status. Li at al.’s study suggested that preoperative infusion of erythropoietin can effectively increase perioperative Hb levels, significantly reduce the number of allogeneic blood transfusions, and does not increase the risk of venous thrombosis (11). Xie et al. pointed out that multiple intravenous infusions of Tranexamic Acid during the perioperative period can effectively reduce hidden blood loss after total hip replacement surgery, alleviate postoperative inflammatory reactions, and shorten hospitalization time (12). For patients with hematological diseases of low erythropoiesis, these treatment measures should be given more attention.

The most common manifestation of CCUS is systemic hematopoietic failure. The absence of granulocytes not only increases the risk of perioperative infection, but also increases the risk of future prosthesis infection (13). For these patients, prevention of perioperative infections is crucial. Hailwood et al. reported that recombinant granulocyte colony-stimulating factor could correct granulocyte deficiency caused by Felty’s syndrome, which was positive and effective in preventing postoperative infections that required joint replacement (14). For patients with agranulocytosis, in addition to active perioperative correction, strict aseptic invasive operations should also be paid attention to avoid possible infectious factors to the greatest extent.

The normal coagulation function of the body relies on a certain number of normally functioning platelets. When the platelet drops to a certain level, it will have an adverse effect on the body’s coagulation function. The disease of CCUS can manifest as isolated thrombocytopenia or thrombocytopenia accompanied by erythroid and granulohematopoietic deficiency (15,16). The decrease of platelet is an important cause of increasing intraoperative bleeding. It should be delayed for joint replacement when the platelets were less than 50×10⁹/L, or taken preoperative platelet transfusion for correction (17). It should be pointed out that the half-life of platelet is relatively short. Therefore, it is necessary to reasonably grasp the infusion time and maximize the hemostatic effect. In addition, regional or intra-spinal anesthesia should be avoided in such patients. During general anesthesia, avoid intubation stimulation that can cause mucosal bleeding and infection (18).

This study reported the surgical case of hip replacement in CCUS patients for the first time, but the sample size was small, the follow-up time was short, and there was a lack of long-term data observation with a large sample size. Nevertheless, we provided treatment recommendations for perioperative orthopedic surgery for patients with hematological disorders and look forward to more clinical research focusing on this issue.

Conclusions

In summary, CCUS patients have a higher risk of bleeding, infection, and anemia, and it is necessary to actively correct the patient’s hematopoietic failure status during the perioperative period. The operation of these diseases should be multidisciplinary to minimize the risk of surgery. After active and effective treatment, joint replacement surgery is feasible for patients with CCUS.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://acr.amegroups.com/article/view/10.21037/acr-24-279/rc

Peer Review File: Available at https://acr.amegroups.com/article/view/10.21037/acr-24-279/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-24-279/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration and its subsequent amendments. Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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