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| CASE REPORT |
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| Year : 2020 | Volume
: 9
| Issue : 3 | Page : 257-259 |
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Gangrene and sepsis in sickle cell disease
Umma Abdulsalam Ibrahim1, Ibrahim Aliyu1, Nuraddeen Lawal2, Aisha Musa Bala2
1 Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University Kano, Kano, Nigeria
2 Department of Paediatrics, Aminu Kano Teaching Hospital, Kano, Nigeria
| Date of Submission | 30-May-2020 |
| Date of Decision | 22-Jul-2020 |
| Date of Acceptance | 30-Jul-2020 |
| Date of Web Publication | 07-Nov-2020 |
Correspondence Address:
Umma Abdulsalam Ibrahim
Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University Kano, Kano
Nigeria
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/sjhs.sjhs_75_20
Infection is common in sickle cell disease (SCD), and consequences of infection such as abscess formation and osteomyelitis are commonly reported events. However, gangrene is an uncommon complication of SCD. A 3-year-old boy with sickle cell anemia presented with fever, bone pains, and gangrene of the digits. Peripheral pulses were palpable. His full blood count showed a progressive increase of the white blood cell count, but the clotting profile was normal, and blood culture yielded Proteus vulgaris. Arterial Doppler scan showed reduced flow in the right ulnar and both dorsalis pedis arteries. X-ray of the upper and lower limbs showed chronic osteomyelitis. Pus aspirate from incision and drainage of anterior tibia abscesses showed Gram-negative cocci in clusters, but the culture was negative. He received blood transfusion, fresh frozen plasma, heparin, and antibiotics. Early recognition and prompt treatment of infection will minimize the development of these devastating complications of SCD.
Keywords: Chronic osteomyelitis, digital gangrene, multifocal abscess, sickle cell anemia
How to cite this article:
Ibrahim UA, Aliyu I, Lawal N, Bala AM. Gangrene and sepsis in sickle cell disease. Saudi J Health Sci 2020;9:257-9 |
| Introduction | |  |
Sickle cell disease (SCD) patients have a defective immune system, which increases their susceptibility to infections. Abscesses and osteomyelitis, which are consequences of infection, are common complications; however, gangrene is an uncommon complication of SCD.
Digital gangrene (DG) is a cause of significant morbidity and mortality, which often requires multiple limb amputations. Mortality rate is reported to be 18%–40%, and survivors have a high frequency of amputation; the literature reports an amputation rate upward of 70%.[1],[2] A wide array of infective and noninfective etiological factors has been linked to DG. It has been described in conditions associated with sepsis, low-flow state, vasospastic conditions, and hyperviscosity syndromes. We, therefore, report a 3-year-old boy with SCD who presented with DG, multifocal abscesses, and chronic osteomyelitis.
| Case Report | | |
A 3-year-old boy with sickle cell anemia presented with fever, bone pains, and gangrene of the digits. The gangrene affected the toes [Figure 1], distal phalanges of the right index and middle fingers, and tip of the left index finger. He was diagnosed to have SCD at 6 months of age. He was on follow-up at another health facility though not regular on follow-up. He had five hospital admissions and had blood transfused on three occasions. He had received Haemophilus influenzae Type B and pneumococcal conjugate vaccines. He had earlier received care for the same complaint at a public health facility a week into the illness. He received ceftriaxone, meropenem, artesunate, pentazocine, intravenous fluids, and additive blood transfusion, without significant improvement, necessitating his referral to our facility.
On examination, he was febrile (39°C), moderately pale, and he had diffuse tender, swelling of the whole upper and lower limbs bilaterally and symmetric darkening of all the toes of the feet. Peripheral pulses were palpable, and abdominal examination revealed no organomegaly. Initial laboratory investigation showed hemoglobin of 5 g/dl, total leukocyte count of 25,600/mm3, and platelet count of 68,000/mm3. The total leukocyte count progressively increased with fall in the hemoglobin from the 1st to 3rd week, but the clotting profile was normal. He had three blood cultures from the 1st to 3rd week: only the first yielded Proteus vulgaris. Arterial Doppler scan showed reduced flow in the right ulnar and both dorsalis pedis arteries. X-ray of the upper [Figure 2] and lower limbs [Figure 3] showed features of chronic osteomyelitis. He received blood transfusion, fresh frozen plasma and heparin, ceftazidime, and azithromycin; other medications were ibuprofen, morphine, and intravenous fluid. Orthopedic surgeon reviewed and suggested disarticulation when the progression has stopped. The incision and drainage of anterior tibia abscesses [Figure 4] was done, and pus aspirate showed Gram-negative cocci in clusters, but the culture was negative. On the 4th week of admission, fever was yet to resolve, ceftazidime was discontinued, and clindamycin was introduced following which the abscesses and fever resolved. He developed an autoamputation of the necrotic digits [Figure 5] and was discharged on the 8th week of admission. Currently, he remains afebrile at his 6-month follow-up visit. | Figure 2: X-ray of the right upper limb showing features of chronic osteomyelitis
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 | Figure 4: Dressing over the anterior tibia after incision and drainage of the tibia abscess
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| Discussion | | |
Our patient developed multifocal abscesses; chronic osteomyelitis and DG. DG is a rare and devastating complication of SCD.
DG is a distal ischemic damage which leads to gangrene of two or more sites in the absence of large vessel obstruction or vasculitis. As such, distal pulses are intact as large vessels are spared.[3],[4],[5] As observed in our patient, the peripheral pulses were intact. The sickle cell gene confers an increased susceptibility to infection and also provokes a cascade of SCD-specific pathophysiologic changes such as chronic inflammation, activation of coagulation cascade, vaso-occlusion, and chronic hemolysis. SCD patients have increased susceptibility to infections, especially encapsulated bacteria as well as Gram-negative organisms such as Streptococcus pneumonia, Staphylococcus, and salmonella species. The increased susceptibility to infection is attributed to defect in the immune system which occurs due to splenic dysfunction, lower serum immunoglobulin level M, sluggish alternative complement pathway activation, defective opsonization, recurrent vaso-occlusions, and infarcts. Commonly reported consequences of infection include abscess formation and osteomyelitis. Osteomyelitis, which can either be acute or chronic, is one of the main bony complications of SCD. Osteomyelitis may result from hematogenous spread or local extension from a contiguous soft-tissue infection or chronic overlying open wounds.[6]
DG results from a low-flow state, which is commonly present in association with a hypercoagulable vasospastic situation, leading to microcirculatory occlusion. It may also result from bacterial endotoxin release and platelet plugging in peripheral arterioles due to vascular collapse and disseminated intravascular coagulation (DIC).[3],[7],[8] Reduced activity of naturally occurring anticoagulants protein C and protein S and inherited hypercoaguable state may also be a contributory factor.
The ischemic changes begin distally and may advance proximally to involve a whole extremity. A more or less prototypical clinical presentation of DG in spite of a large number of etiological associations is suggestive of DIC as the final common pathway of its pathogenesis.[3] DIC might be associated with 85%–100% of cases of DG.[3],[5] DIC most commonly occurs due to sepsis, and pneumococcus is the most common organism responsible.[7] Other infective organisms such as Staphylococcus aureus, Streptococcus pyogenes, Klebsiella pneumoniae, Escherichia coli, Salmonella paratyphi, P. vulgaris, Proteus mirabilis, Pseudomonas, and Enterococcus faecalis have been implicated as the causative agents of DG.[4] Blood culture isolates from our patient yielded P. vulgaris. Although our patient initial coagulation studies were normal, a repeat screening for features of DIC should have been undertaken as studies have shown that laboratory features of DIC may be absent in the early course of the disease but may evolve later in the course of the disease.[2],[5] However, due to financial constraints, the coagulation studies were not repeated. DIC should be corrected as appropriate; its cause should be found out and treated aggressively. Management of DIC should be guided by basic tests of coagulation.[1],[2] If bleeding is the predominant feature, depleted coagulation factors are replaced. On the other hand, in cases where thrombosis is predominant, anticoagulants such as heparin are used.
| Conclusion | | |
DG is a rare and devastating complication of SCD, which often leads to multiple limb amputation. DIC is an almost universal finding and is probably the final common event in the microvascular insult that gives rise to the prototypical clinical features. Awareness, early recognition of infection and acralcyanosis, prompt management of DIC and underlying condition, hemodynamic stabilization, and use of anticoagulants may halt the progression of the disease and minimize the development of these devastating complications of SCD.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the caregivers have given their consent for the patient's images and other clinical information to be reported in the journal. The caregivers understand that the patient's name 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.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
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