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Abstract
Introduction
Case Report
Discussion
Conclusion
References
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CASE REPORT
Year : 2019  |  Volume : 20  |  Issue : 1  |  Page : 35-38
 

Anesthetic repercussions in a child with aplastic anemia


Department of Anesthesia, VMMC and Safdarjung Hospital, Delhi, India

Date of Submission07-Sep-2018
Date of Acceptance31-Oct-2018
Date of Web Publication6-May-2019

Correspondence Address:
Dr. Sapna Bathla
Department of Anesthesia, VMMC and Safdarjung Hospital, Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/TheIAForum.TheIAForum_51_18

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  Abstract 


Aplastic anemia (AA) is a rare hematologic disease and a unique example of bone marrow failure syndromes. The disease presents with clinical features of reduction in all blood cell counts, i.e., anemia, infections, and hemorrhage. We are reporting the successful anesthetic management of a case of 6-year-old, 20 kg male child, diagnosed case of AA operated for a fungating lesion (osteomyelitis) in the right wrist.


Keywords: Anesthesia, aplastic anemia, pediatric


How to cite this article:
Karim W, Bathla S, Bajaj JK, Choudhuri P. Anesthetic repercussions in a child with aplastic anemia. Indian Anaesth Forum 2019;20:35-8

How to cite this URL:
Karim W, Bathla S, Bajaj JK, Choudhuri P. Anesthetic repercussions in a child with aplastic anemia. Indian Anaesth Forum [serial online] 2019 [cited 2019 Nov 13];20:35-8. Available from: http://www.theiaforum.org/text.asp?2019/20/1/35/257681





  Introduction Top


Aplastic anemia (AA) is a rare hematologic disease and a unique example of bone marrow failure syndromes. AA is characterized by reduction or absence of hematopoietic precursors in the bone marrow due to injury to the pluripotent stem cell.[1] Patients present with clinical features of reduction in all blood cell counts, i.e., anemia, infections, and hemorrhage. In general, infections are bacterial, but invasive fungal infections tend to be fatal, especially in patients with prolonged and severe neutropenia. Anemic patients present with easy fatigability. Thrombocytopenia causes easy bruisability and spontaneous bleeds, which can be life-threatening in the perioperative period.[2]


  Case Report Top


A 6-year-old, 20 kg male child, diagnosed case of AA was scheduled for surgery of a fungating lesion (osteomyelitis) on the right wrist [Figure 1]. The patient was diagnosed with the disease 18 months ago when he developed uncontrolled bleeding after a dental extraction, following which he was referred to our tertiary care center. After the diagnosis was made, the patient was put on cyclosporine and prednisolone treatment. He developed cyclosporine-induced hypertension for which amlodipine and enalapril were started. The patient was on prophylactic antibiotics and antifungals. He had a platelet count of 45,000 and was transfused single donor apheresis platelet unit (SDAP), but the count continued to spiral downwards. Preoperative investigations showed Hb 9.5 g/dL, platelets – 30 × 109/L, thin-layer chromatography – 0.9 × 109/L, and normal electrocardiography and renal functions. Preoperative transfusion of 2 units platelet-rich plasma (PRP) was given and 1 unit PRP and 1 unit Packed cells were arranged for the intraoperative period.
Figure 1: Fungating lesion in the right wrist

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After getting informed consent from attendant, the patient was taken up for surgery. In operation theatre (OT), all routine monitors were attached which showed heart rate-79/min blood pressure (BP) – 96/54 mm hg, SpO2– 100% on room air, and relative risk – 22/min. General anesthesia with supraglottic device was planned avoiding regional blocks in view of low platelet count. As an intravenous (IV) cannula was already in situ, the patient was premeditated with midazolam 0.5 mg and fentanyl 20 mcg. Anesthesia was induced with propofol 40 mg, after check ventilation, vecuroniun 2 mg was given, and I Gel size 2 was inserted in a single attempt, and the patient was put on controlled ventilation after confirming the adequate seal. Anesthesia was maintained with isoflurane with minimum alveolar concentration 1% and O2 and N2O 50:50% in combination. Multimodal analgesic regimen was chosen for analgesia with paracetamol 300 mg and fentanyl while nonsteroidal anti-inflammatory drugs (NSAIDs) were avoided. The surgeons were advised to defer the use of routine tourniquet and exsanguination was achieved by limb elevation and BP cuff inflation. After the excision of the surgical lesion, bleeding was controlled using hemostatic agent feracrylum, locally and IV tranexamic acid 200 mg. Total blood loss was 30 ml. Rest of the surgery went uneventful and I Gel was removed after adequate reversal. Postoperative analgesia was provided with paracetamol and tramadol, and tranexamic acid was given for 3 days' postoperatively.


  Discussion Top


AA is a rare disease with the overall incidence of is 2.34 per million inhabitants per year. The incidence increases with age, and the 2-year mortality rate with supportive care alone for patients with Severe AA (SAA) or Very Severe AA (VSAA) approaches 80%.[3] AA is a syndrome of bone marrow failure characterized by peripheral pancytopenia and marrow hypoplasia in which normal hemopoietic marrow is replaced by fat cells. AA, by definition includes at least two of the following: (i) hemoglobin <10 g/dL, (ii) platelet count <50 × 109/L, or (iii) neutrophil count <1.5 × 109/L3.

AA is classified into nonsevere (NSAA), SAA and VSAA according to the classification given by Camitta et al., and Bacigalupo et al.[4],[5]

SAA: Bone marrow cellularity <25% or 25%–50% with <30% residual hemopoietic cells and two out of three of the following:

  1. Absolute neutrophil count (ANC) <0.5 × 109/L
  2. Platelets <20 × 109/L
  3. Reticulocyte count <20 × 109/L.


VSAA: As for severe but ANC <0.2 × 109/L.

NSAA: Patients not fulfilling the criteria for severe or very severe AA.

Clinical presentation of AA varies with the type of cell line depressed. Anemia or thrombocytopenia are usually first to manifest. Anemia presents as failure to thrive, pallor, palpitations, dyspnea on exertion, and thrombocytopenia usually presents with petechiae, purpura, epistaxis, gingival bleeding, or ecchymoses. Fever, repeated respiratory tract infections and sepsis may be present as a late feature of neutropenia.[6] A complete blood count, leukocyte total and differential counts, reticulocyte count and a bone marrow aspirate and biopsy help clinch the diagnosis.

Treatment

The treatment of AA is divided into specific and supportive. Specific treatment has two modalities - allogenic bone marrow transplant and immunosuppression. Hematopoietic stem-cell transplantation or bone marrow transplantation (BMT) is the treatment of choice for young patients who have a matched sibling donor.[7] Immunosuppressants are aimed at suppressing the destruction of blood stem cells that occurs in the disease. Immunosuppression with either anti-thymocyte globulin and cyclosporine or high-dose cyclophosphamide is an effective therapy for patients who are not suitable for BMT owing to age or lack of a suitable donor. Despite their desired effects, immunosuppressants decrease the immunity and reduce threshold for infections and cancers. This patient might have developed osteomyelitis of the wrist joint as a consequence of immunosuppression. Besides this, cyclosporine has side effects such as nephrotoxicity, gum hypertrophy, hirsutism, tremor, hypertension, renal dysfunction, dyselectrolytemia-magnesium or potassium loss, and liver inflammation.[8]

Our patient was found hypertensive after being initiated on cyclosporine therapy for which amlodipine was started as an antihypertensive therapy. Granulocyte colony-stimulating factor (GCSF) is an endogen resulting in spurt of granulocytes. GCSF is used in certain centers despite inconclusive evidence of its usage. As levels of the endogenous growth factors are elevated in patients' blood, GCSF therapy frequently fails in such patients.

Steroids are given in adjunct to immunosuppressive therapy, often in high doses and for long periods which may lead to invasive fungal infections and iatrogenic Cushing syndrome.[7],[9]

To prevent infections, antimicrobial prophylaxis for Pneumocystis carinii is administered using pentamidine, dapsone, and atovaquone. Antifungal treatment is also started in patients manifesting fungal infections. Nephrotoxic agents should be dose adjusted in patients having cyclosporine-induced kidney dysfunction.[7]

For the management of such cases, a multidisciplinary approach involving surgeons, anesthesiologist, and pediatric hematologist is needed. Easy and timely availability of blood and blood components by virtue of a well-equipped blood bank is essential to ensure a successful outcome.

General anesthesia was preferred over regional anesthesia in this case due to low platelet count. It is easier to assess the hemodynamics and manage blood loss when a patient is anesthetized and well oxygenated. It is imperative to take aseptic precautions for all anesthetic maneuvers including IV cannulation and endotracheal intubation to avoid infection. Bacterial and viral filters should be used in the breathing circuits. For the same reason, perioperative antibiotic cover is essential.

Laryngoscopy and invasive maneuvers should be gentle to avoid trauma, hence experienced anesthesiologist should perform these procedures. There is a risk of bone marrow suppression and bleeding with the use of nitrous oxide and inhalational agents respectively, keeping this in mind air was used for maintenance of anesthesia.[10] The use of tourniquet for intraoperative bleeding is avoided to prevent pressure hematomas. Surgical bleeding can be controlled by means of exsanguinations of limb, pressure bandage, pharmacological measures such as tranexamic acid epsilon-aminocaproic acid and feracrylum for local application.[11] All pressure points should be well padded to avoid pressure injuries leading to hematomas.

In anticipation of the intraoperative bleeding, adequate blood and blood products should be readily available. Red cell transfusion is essential for patients with AA to maintain a safe hemoglobin level (>8 g/dL in the absence of comorbidities and 10 g/dL in patients with cardiac disease). Frequent erythrocyte transfusions lead to alloimmunization against erythrocytic antigens, and iron overload hence should be avoided. The threshold for prophylactic platelet transfusion is between 10 and 20 × 109/L in patients having additional risk factors for bleeding. In our patient, platelet threshold was taken as 20,000/dL as the patient was febrile and septic. Blood products should be irradiated to prevent transfusion-associated graft-versus-host disease and filtered to reduce the incidence of viral infections and prevent alloimmunization. All transfusions and any transfusion reactions must be documented. No family member should be used as a blood product donor until it has been determined that a transplant will not be performed. SDAPs are preferred to reduce the frequency of antibody formation.[12],[13]

Postoperative analgesia is imperative in these patients as pain can lead to diaphragmatic splinting, retention of secretions and subsequent lung infections, inadequate tidal volume generation. Thrombocytopenia renders them unfit for regional blocks, and NSAIDs are contraindicated to prevent any further drop in platelets, therefore after preoperative counseling, paracetamol with tramadol was given.

The decision for surgery should be made carefully and surgery undertaken only if the patient has a life-threatening or debilitating condition requiring surgical intervention.


  Conclusion Top


Interdisciplinary team approach with good coordination, cooperation, and communication between orthopedicians, anesthesiologists, and hematologists in the management of a patient with AA was the key to success. Efficient blood bank services are essential for a positive outcome in case of any surgical intervention in such patients.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Singh P, Sinha A. Aplastic anemia – A quick review. J Cancer Prev Curr Res 2017;7:251.  Back to cited text no. 1
    
2.
Young NS. Acquired aplastic anemia. Ann Intern Med 2002;136:534-46.  Back to cited text no. 2
    
3.
Montané E, Ibáñez L, Vidal X, Ballarín E, Puig R, García N, et al. Epidemiology of aplastic anemia: A prospective multicenter study. Haematologica 2008;93:518-23.  Back to cited text no. 3
    
4.
Camitta BM, Thomas ED, Nathan DG, Santos G, Gordon-Smith EC, Gale RP, et al. Severe aplastic anemia: A prospective study of the effect of early marrow transplantation on acute mortality. Blood 1976;48:63-70.  Back to cited text no. 4
    
5.
Bacigalupo A, Hows J, Gluckman E, Nissen C, Marsh J, Van Lint MT, et al. Bone marrow transplantation (BMT) versus immunosuppression for the treatment of severe aplastic anaemia (SAA): A report of the EBMT SAA working party. Br J Haematol 1988;70:177-82.  Back to cited text no. 5
    
6.
Jennison T, Hardwicke J, Brewster M. An unusual presentation of aplastic anaemia: Compartment syndrome. J Surg Case Rep 2014;2014. pii: rjt095.  Back to cited text no. 6
    
7.
Dezern AE, Brodsky RA. Clinical management of aplastic anemia. Expert Rev Hematol 2011;4:221-30.  Back to cited text no. 7
    
8.
Scheinberg P, Young NS. How I treat acquired aplastic anemia. Blood 2012;120:1185-96.  Back to cited text no. 8
    
9.
Brodsky RA, Jones RJ. Aplastic anaemia. Lancet 2005;365:1647-56.  Back to cited text no. 9
    
10.
Carmel R, Rabinowitz AP, Mazumder A. Metabolic evidence of cobalamin deficiency in bone marrow cells harvested for transplantation from donors given nitrous oxide. Eur J Haematol 1993;50:228-33.  Back to cited text no. 10
    
11.
Lahoti BK, Aggarwal G, Diwaker A, Sharma SS, Laddha A. Hemostasis during hypospadias surgery via topical application of feracrylum citrate: A randomized prospective study. J Indian Assoc Pediatr Surg 2010;15:87-9.  Back to cited text no. 11
[PUBMED]  [Full text]  
12.
Marsh J, Socie G, Tichelli A, Schrezenmeier H, Hochsmann B, Risitano AM, et al. Should irradiated blood products be given routinely to all patients with aplastic anaemia undergoing immunosuppressive therapy with antithymocyte globulin (ATG)? A survey from the European Group for Blood and Marrow Transplantation Severe Aplastic Anaemia Working Party. Br J Haematol 2010;150:377-9.  Back to cited text no. 12
    
13.
Estcourt LJ, Birchall J, Allard S, Bassey SJ, Hersey P, Kerr JP, et al. Guidelines for the use of platelet transfusions. Br J Haematol 2017;176:365-94.  Back to cited text no. 13
    


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