PEDIATRIC DENTISTRY/Copyright © 1981 byThe American Academy of Pedodontics/Vol. 3, No. 2 CAS

Periodontal changes associated with chronicidiopathic neutropenia

Kenneth L. Kalkwarf, DD$, MSDennis P. Gutz, DDS, MS

AbstractThis case report focuses on the periodontal status of a

young patient with chronic idiopathic neutropenia. A four-year clinical course is presented. Chronic idiopatin’cneutropenia is a relatively rare blood dyscrasiacharacterized by a severe decrease in the number ofcirculating neutrophils. Young individuals exin’biting tinsproblem experience rapid destruction of periodontalstructures. A review of related literature reveals that the fewdiscussions of dental symptoms available relate commonfindings: exaggerated gingivitis, premature exfoliation of theprimary dentition and recurrent occurrence of aphthous-type lesions. While some mechanisms explaining the lack ofcirculating neutrophils have been postulated, the exactetiology of chronic idiopathic neutropenia is unknown.Dental therapy for individuals exin’biting tins conditionshouM be aimed at the reduction of local inflammation bycareful and frequent removal of local irdtational factors.Great care shouM be employed during dental therapy toreduce the possibility of bacteremias.

and respiratory tract. Several cases7-11,~,~.4~-57.4.s~ have dis-cussed the response of the gingiva and periodontalsupporting apparatus during the neutrophil disorder.Severe gingivitis, oral ulcers and destruction of thealveolar crest have been consistently noted.

Suggested therapy for children exhibiting thishematologic phenomena has included cysteine, ~ bloodtransfusions/ pyridoxine hydrochloride (vitamin B6),~.~ plasma transfusions,s transfusion with packed whitecells, ~ non-specific fever therapy/ splenectomy,u,~3

cortisone, 41,~.u testosterone,5 and somatotropic hor-mone? Only antibiotic therapy has had predictablesuccess in control of the systemic sequela.’s,~a.4~ Newermethods of granulocyte transfusion appear to exhibitsignificant promise in future therapy regimes?~

The purpose of this paper is to present a case reportof a child exhibiting chronic neutropenia, discuss clas-sification of such neutropenias and review dental man-agement for children with such problems.

Introduction

Neutropenia is characterized by a severe decreasein the number of circulating neutrophils in the peri-pheral blood vessels. Classically, neutropenia occurs inadults, typically middle-aged women, and is a result ofspecific drug injection, radiation or severe infection;

Several cases of infants and children exhibitingsevere neutropenias of questionable or idiopathicorigin have been reported in the literature. Due todifferences observed in onset, severity, symptoms andetiology, a number of names have been used to de-scribe the clinical entities: infantile congenital agranu-locytosis, ~ cyclic neutropenia, TM chronic benignneutropenia, m~ reticular dysgenesia,1~,~ congenitalneutropenia,~*= infantile aneutrocytosis," chronic idio-pathic neutropenia~ and others. ~-s~ All children re-ported a history of persistent infections of the skin

Accepted: December 2, 1980

Medical History

The patient, a slight, well nourished 31/~-year-oldcaucasian female, in no apparent distress, was referredto the Periodontics Department, College of Dentistry,University of Nebraska Medical Center, for evaluationand treatment of her gingival condition. A medicalhistory revealed that initial problems were noted atage 21/~ weeks, when she developed an intense papularrash in the diaper area. A diagnosis of staphylococcaldermatitis was made and the patient was treated withampicillin and oxacillin sodium. The rash cleared, butrecurred two weeks following discontinuance of theantibiotics. The patient also experienced numerousearly bouts of respiration difficulty and frequentfebrile episodes, with temperatures ranging from 101-103° F. Each illness was treated with antibiotic ther-apy. The clinical picture recurred with cessation ofeach course of medication.

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Vo|. 3, No. 2

The patient was evaluated at age three months anda diagnosis of hypogammaglobulinemia was made.She was treated with two injections of gammaglobu-lin. Multiple complete blood counts were completedduring the course of her initial therapy. At no timewas her granulocyte count above 3%.

At age five months the patient was referred to theUniversity of Nebraska Medical Center for a hema-tologic evaluation. Laboratory data obtained at thattime was as follows:

Hemaglobin: 11.5 Platelets: 552,000WBC: 10,900 Urinalysis: normalDifferential: 76% Lymphocytes IgG: 630

20% Monocytes IgA: 453% Eosinophils IgM: 97l%Basophils9% Neutrophils

A repeat blood count was comparable to the first. Abone marrow examination was reported to be consist-ent with congenital neutropenia. A diagnosis of con-genital neutropenia, specifically of the congenitalinfantile agranulocytosis form, was made.

Following the diagnosis, the patient experiencednumerous dermatologic infections that were treatedwith antibiotic therapy. At age two, severe otitis re-quired lancing of the typanic membranes. Ampicillintherapy was initiated at that time and continued inmultiple two week courses.

Family History

Outside of a family history of allergic disorders, nosignificant familiar characteristics could be identified.Hematologic evaluations of the patient's parents werewithin normal limits.

Dental Findings

Intital periodontal examination revealed swollen,edematous gingiva. A distinct granulomatous collarwas evident at the cervical region of the teeth. It wascrimson red in color and bled easily when manipulated(Figure 1). No evidence of perioral or other lesionswithin the oral cavity or pharyngeal region waspresent. Gingival recession had occurred in the ante-rior regions, resulting in exposed root surfaces. Theginival tissue was very tender and the patient resistedattempts at gentle probing. No clinical evidence of ac-tive carious lesions was detected. Moderate accumula-tions of plaque were present. The mother noted thatthe patient frequently complained of a "sore mouth"and resisted attempts at toothbrushing. Radiographicexamination revealed exaggerated alveolar bone loss,particularly in the anterior regions of the mouth (Fig-ures 2 and 3). No radiographic evidence of active car-ies was found.

Figure 1. Age three years, six months, mild plaque accumulation. Agranulomatous collar forms the gingival margin around each tooth.Exaggerated gingival recession is evident, particularly in the ante-rior region.

3y,6m

4y, 6m

6y, 9mFigure 2: Serial bitewing radiographs.

The importance of plaque control was stressed tothe mother. She was instructed in disclosing andcleaning her daughter's mouth with an extra softnylon brush, being careful to remove all traces of vis-ible plaque. An emollient was prescribed to providepallative relief to local regions devoid of epithelium. Athorough prophylaxis was performed and the patientwas placed on a frequent maintenance regimen. Thepatient's mother was told to call if any change in oralsymptoms was noticed.

The patient was seen at one to three month inter-vals over the next four years. At maintenance appoint-

190CHRONIC IDIOPATHIC NEUTROPENIAKalkwarf and Gutz

5y, 0 m

6y, Om

6y ,9mFigure 3: Serial anterior radiographs.

ments, plaque control was evaluated and discussedwith the patient and her parents. Gingival contour,color and consistency were evaluated. Gentle debride-ment and polishing were accomplished as required.Kodachromes were obtained at six-month intervals fordocumentation. Bitewing and anterior radiographswere obtained yearly to evaluate osseous changes (Fig-ures 2 and 3).

During the four years of observation, the patient'splaque control varied from fair to poor. From conver-sations with her mother it was determined that gin-gival inflammation periodically progressed to a pointwhere use of even a soft toothbrush was very uncom-fortable. During these periods, the parents would notinsist that cleaning be performed and massiveamounts of bacterial plaque would accumulate, result-ing in increased gingival swelling and edema (Figure4). The granulomatous collar remained present at eachobservation period, being more evident during someperiods than others (Figures 1, 5 and 8).

Progressive gingival recession eventually resulted inadvanced root exposure throughout the primary den-tition. This was especially evident in the anterior re-gions of the mouth (Figures 5 and 6). Instrinsic stainingbecame readily apparent in both the primary and per-

Figure 4: Age four years, moderate plaque, gingival edema and

swelling. Gingival architecture in the anterior regions is becoming

quite distorted. Granulomatous collar is not as evident.

Figure 5: Age four years, six months, moderate plaque accumula-

tion. Recession is more pronounced in the anterior region. Drifting

of the mandibular anterior teeth is becoming noticeable.

Figure 6: Age five years, mild plaque accumulation. Recession and

staining are more prominent. The mandibular incisors are extremely

mobile and exhibit evidence of drifting.

manent dentition (Figures 5, 6, and 7). Clinical evi-dence of active caries was not identified at any exami-nation. Soft tissue lesions, appearing to be aphthous-type ulcerations were occasionally present in the oraland perioral regions (Figure 9). Radiographic evalua-tions revealed progressing destruction of the alveolarbone about the primary dentition. This was especiallyevident about the anterior teeth and in the furcationregions of the primary molars (Figures 2 and 3).

The mandibular primary incisors were exfoliated at5 years, 7 months. Eruption of the permanent incisors

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Figure 7: Age six years,plaque accumulation moder-

ate. Maxillary incisors are mo-

bile and show evidence of

drifting. The deciduous mandi-

bular incisors were exfoliated

at age five years, seven

months.

followed a normal pattern, although chronologicallyadvanced. Alveolar support about the permanentteeth is within the normal limits through age seven.

Discussion

Several systemic conditions in children can initiatethe oral manifestations of gingival inflammation, al-veolar bone loss and mucosal ulceration. A differentialdiagnosis for a child presenting with these conditionsmust include such entities as juvenile periodonti-tis,56-57 Papillon-Lefevre syndrome,58 histiocytosis X,59

Chediak-Higashi syndrome,60'61 acatalasia,62 chronicgranulomatous disease,63 hypophophatasia,64 diabetes,65

leukemia,65 and neutropenia.Juvenile periodontitis and Papillon-Lefevre syn-

drome may be confirmed by the pattern of onset,clinical and radiographic features and lack of distin-guishing laboratory features. Biopsy will confirm his-tiocytosis X. Chediak-Higashi syndrome exhibitsidentifying characteristics in peripheral leukocytesand lymphocytes as well as peculiar eosinophila inclu-sion bodies in the myeloid cells of the marrow. Aca-talasia may be identified by clinical or hemotologicevaluation for blood catalase activity. Chromic granu-lomatous disease can be positively diagnosed by thequalitative nitro blue tetrazolium test. Hypophospha-tasia may be confirmed by measurement of serum al-kaline phosphatase. Hematologic evaluation will posi-tively identify diabetes, leukemia or neutropenia.

Neutropenic conditions in childhood are separatedinto various classifications based upon clinicalsymptoms, age at onset, duration, white cell counts,immune function, familial tendencies and bone mar-row alterations. Cyclic neutropenia is the mostcommon form of granulocyte alteration exhibiting per-iodontal changes.711 In this disease, a predictably re-current diminution of neutrophils occurs as a result ofsuppression or maturation arrest. The phenomenonoccurs for five to seven days at regular 21-day inter-vals. The etiology of the process is unknown. In thetime periods between these suppressions, the whiteblood cell counts increase, but neutrophil counts sel-dom exceed 50% of the total leukocyte population. Al-though the clinical symptoms exhibited in this case

Figure 8: Age six years, three months, plaque accumulation moder-

ate. The deciduous, maxillary, incisors were exfoliated at age six

years, '/2 month. Decalcification of the remaining deciduous teeth

is clinically visible, but there is no evidence of an active cariousprocess.

were similar to those reported for cyclic neutropeniaby Telsey,10 and Wade,11 the persistence of the neutro-penia exhibited by our patient does not allow dupli-cate classification.

Classifications such as infantile genetic agranulocy-tosis2*43'44 as well as genetic, familial or hereditaryneutropenias3742'4™9 were ruled out because of the appar-ent lack of hereditary ties. The term reticular dys-genesia161766 cannot be utilized because the patientexhibits no evidence of an immunodeficiency. Theterm chronic benign neutropenia131546 was not appliedbecause this patient shows no evidence of spontaneousremission.

Several individuals19-20-31 •" have described cases ofneutropenia in children which they labeled as chronicidiopathic neutropenia (granulocytopenia). The condi-tion was so named because of the clinical course. Apersistent neutropenia was present from birth with noapparent etiology; no familial tendencies were discov-ered. It differed from chronic benign neutropenia inthat the children were followed for several years withno documentation of self-limitation. Clinicalsymptoms consisted of persistent, recurrent infections,including gingivitis, throughout the patient's lifetime.Laboratory tests revealed severely depressedneutrophil counts with accompanying rises in thenumber of monocytes, lymphocytes and eosinophils.Bone marrow specimens revealed that granulopoiesiswas active and orderly to the myelocyte level. Meta-myelocytes, bands and segmented forms were rare. Nodecrease in immune functions was detectable. Ther-apy modalities other than antibiotics to control therecurrent episodes of infection were not successful.The condition apparently varied in severity from indi-vidual to individual. Some of the patients may surviveinto adolescence with suitable antibiotic therapy. Thispatient meets all those criteria and can be diagnosedas having chronic idiopathic neutropenia.

Her dental findings are similar to other reportedneutropenia cases.1525464752 While periodontal destruc-

192CHRONIC IDIOPATHIC NEUTROPENIAKalkwarf and Gutz

Figure 9: Age six years, six

months. An apthous-type le-

sion present in the region of

the right commissure.

tion, marked gingival recession, tooth discolorationand moderate to heavy accumulations of plaque werepresent, there was never any evidence of carious activ-ity during the four years of follow-up. Tooth discolor-ation was primarily a result of intrinsic staining, prob-ably due to antibiotic ingestion during formative peri-ods and extrinsic staining due to plaque control at-tempts that were below acceptable levels. The lack ofcarious activity in this patient and in others withneutropenic conditions"2545" is difficult to explain. Itmay be that the relative lack of certain inflammatorycells alter the microbial makeup to one incapable ofinitiating a carious process.

Infections in children with chronic neutropenic con-ditions are common.6"8 With the lack of polymorpho-nuclear leukocytes in the circulation, it is assumedthat the increased susceptibility to infection is due tothe diminished phagocytic clearing of bacteria. Thepatients commonly exhibit a compensatory increase ineosinophils and monocytes in the bone marrow andperipheral blood. While these cells are also phagocytic,it has been shown that they are inferior to neutrophilsin their ability to clear bacteria.6970'71 Generally theinfections reported in the medical literature are ofdermal or upper respiratory origin. It stands to reasonthat the oral cavity, with its constant exposure todental plaque, would be a common site for infectionsto appear.

While the exact role of the neutrophil in the protec-tion of periodontal tissues has not been delineated,several investigators have determined that the cellmay pass through junctional epithelium and reside inthe gingival sulcus in a viable state.7273 Decreased num-bers of neutrophils have also been associated with de-velopment of gingivitis.74 Recent work has relateddecreased neutrophil function with increased suscepti-bility to certain periodontal diseases.7576 It appearslogical that the exaggerated periodontal destructionseen in patients with chronic idiopathic neutropenia isa result of decreased protection by the neutrophil.

Previous reports24'45 mention that the severe alveolarbone loss occurring around the primary teeth does notappear to interfere with the eruption pattern of thepermanent teeth. Other observations,154750 that follow

the children to a later age, report eventual alveolarbone destruction about the permanent teeth as a com-mon sequela. This patient has exhibited a normaleruption sequence of the permanent dentition. Theeruption schedule has been accelerated, probably dueto the early loss of some primary teeth secondary toaccentuated alveolar bone loss. It is expected that al-veolar destruction about her permanent teeth willoccur with time.

This patient, as well as others2547 exhibited severalepisodes of recurrent aphthous lesions (Figure 9). Suchproblems have been noted to occur frequently, butthere doesn't appear to be a clear relationship withthe circulating neutrophil count.77

With the advent of more sophisticated antibiotictherapy, a greater number of children exhibitinghematologic disorders are surviving infections and re-quiring dental therapy. While some children withneutropenic conditions require no special managementduring dental therapy, many have an increased sus-ceptibility to all infections, including periodontal dis-ease. It is imperative that practicing dentists have athorough knowledge of the dental management regi-mens required by these patients.

Mishkin et al.,25 describe, in great detail, the biora-tionale for dental management of neutropenic pa-tients. Although caries activity does not appear to bea major problem for individuals with chronic idio-pathic neutropenia, daily stannous fluoride gel is rec-ommended to retard the possibility of tooth loss ordental infection secondary to a carious lesion. Stan-nous fluoride application has also been shown to becapable of reducing plaque formation and potentiallyuseful in the control of early periodontal disease.78 Astrict oral hygiene program, complete with scaling andprophylaxis at short-term intervals, is suggested todecrease the intensity of gingival inflammation andslow the rate of alveolar bone loss. Maximum precau-tions, including antiseptic irrigation and antibioticcoverage, prior to tissue manipulation is suggested toreduce the possibility of bacteremias and postopera-tive infections.

Conclusion

Chronic idiopathic neutropenia is characterized bya severe decrease in the number of circulatingneutrophils. The condition is characterized by oralsymptoms consisting of: oral ulceration, gingivalinflammation and rapidly advancing alveolar bonedestruction.

It is important that the practicing dentist be awareof the dental ramifications exhibited by individualswith this disorder and be knowledgeable concerningtheir management.

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Vol. 3, No. 2193

Dr. Kalkwarf is associate professor and director of graduatestudies in periodontics, and Dr. Gutz is associate professor and act-ing chairman of pedodontics, University of Nebraska Medical Cen-ter, College of Dentistry, Lincoln, Nebraska 68583. Requests forreprints should be sent to Dr. Kalkwarf.

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Pin Oak, Iowa John B. Ferguson

[Editor’s Note: Beginning with this issue we will be publishing member’s photographs in Pediatdc Dentistry. Though anysubject matter will be considered, general, scenic photographs (as above) will be favored. Submissions from all membersare invited and will be used on a space-available basis.]

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