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  Table of Contents    
CASE REPORT  
Year : 2018  |  Volume : 61  |  Issue : 2  |  Page : 284-286
Fatal meningococcal septicemia without meningeal signs, contribution of the peripheral smear in diagnosis: Report of a case


1 Department of Pathology, Command Hospital Air Force, Bengaluru, Karnataka, India
2 Department of Dermatology, Command Hospital Air Force, Bengaluru, Karnataka, India

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Date of Web Publication20-Apr-2018
 

   Abstract 


Acute meningococcemia is characterized by extensive purpurae consisting of both petechiae and ecchymoses. This condition can be rapidly fatal without treatment due to shock and severe consumptive coagulopathy. We report a case of fatal meningococcal septicemia in a military recruit who presented with fever and associated rapidly progressive purpuric rash (purpura fulminans) without any meningeal signs. Evaluation revealed evidence of disseminated intravascular coagulopathy and multiorgan failure. Diplococci were demonstrated in peripheral blood neutrophils and monocytes. On autopsy, extensive hemorrhages were found in both adrenals, lungs, liver, skin, and kidneys with secondary hemophagocytic lymphohistiocytosis in bone marrow. This report highlights useful information obtained from examination of peripheral blood smear in purpura fulminans.

Keywords: Meningococcemia without meningitis, peripheral smear, postmortem, purpura fulminans

How to cite this article:
Mutreja D, Moorchung N, Manasa S J, Varghese J. Fatal meningococcal septicemia without meningeal signs, contribution of the peripheral smear in diagnosis: Report of a case. Indian J Pathol Microbiol 2018;61:284-6

How to cite this URL:
Mutreja D, Moorchung N, Manasa S J, Varghese J. Fatal meningococcal septicemia without meningeal signs, contribution of the peripheral smear in diagnosis: Report of a case. Indian J Pathol Microbiol [serial online] 2018 [cited 2020 Jul 7];61:284-6. Available from: http://www.ijpmonline.org/text.asp?2018/61/2/284/230539





   Introduction Top


In the background of overwhelming sepsis due to various infectious agents, namely, meningococci, pneumococci, staphylococci, Haemophilus influenzae and varicella, rapid onset of petechiae, and ecchymoses illustrates an infrequent disorder termed purpura fulminans.[1],[2]

We present the postmortem findings of a military recruit, who died of purpura fulminans secondary to severe meningococcemia and disseminated intravascular coagulopathy (DIC) in the absence of meningeal involvement. The diagnosis came to light when diplococci were demonstrated in peripheral blood neutrophils and monocytes when evaluating the case for a possible acute promyelocytic leukemia and were later confirmed on culture and histopathology. This report highlights careful examination of peripheral blood smear in purpura fulminans.


   Case Report Top


A 21-year-old military recruit, presented with a history of high-grade fever associated with chills and rigors of 2 days duration. There was a history of nonitchy skin rash appearing initially over lower limbs and later appearing over entire body including palms, soles, scalp, and genital area within 4 h of onset. The patient also complained of passage of reddish urine with reduced urine output since morning. There was associated a history of cough, generalized weakness with joints pains, and headache over 1 week. No history of recent travel, drug intake, insect, or animal bite were elicited.

Examination revealed a well-oriented individual with tachycardia, (pulse - 102/min), blood pressure of 130/78 mmHg, with tachypnea, dry-coated tongue, and facial puffiness. Dermatologic examination showed multiple well-defined purpurae coalescing to form polysized ecchymoses over scalp, face, chest, abdomen, extremities, buttocks, palms, and soles [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d. Systemic examination revealed enlarged liver 2 cm below costal margin. Meningeal signs were absent. The patient was admitted with a provisional diagnosis of sepsis with multiorgan dysfunction with possibilities of rickettsial fever, meningococcemia, and dengue hemorrhagic fever. He was managed with injection ceftriaxone 2 g 12 h, capsule doxycycline, and supportive measures. Investigations revealed bicytopenia (total leukocyte count: 2060/μL, platelet count: 17,000/μL) with deranged liver and renal function, elevated lactate dehydrogenase (763 IU), and creatine kinase (1420 IU). A careful evaluation of the peripheral smear revealed the presence of intracytoplasmic diplococci in monocytes and neutrophils [Figure 2]a and [Figure 2]b.
Figure 1: (a-d) Multiple well-defined purpurae coalescing to form polysized ecchymoses over face, chest, extremities, palms and soles

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Figure 2: (a and b) Peripheral smear showing intracytoplasmic diplococci in monocytes and neutrophils (Leishman Giemsa, ×1000); (c) Fibrin thrombi in arterioles with presence of bacterial colonies within blood vessels (H and E, ×400)

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Hematologic evidence of DIC was seen (prothrombin time: Test-37 s/control 12.5 s, partial thromboplastin time: Test-151 s/control 35 s, fibrinogen 50 mg/dl, fibrin degradation products >3200). Routine urine showed 4+ proteins with virus, hepatitis B and C, rickettsial fevers, dengue, and malaria were negative. Sickle cell screen was negative. Chest roentgenogram showed features of adult respiratory distress syndrome. The condition of the patient rapidly worsened with the appearance of hypotension, altered sensorium, hypoxemia and anuria. He was intubated and put on mechanical ventilator support; however, he died 12 h after admission.

An autopsy revealed congested kidneys and heavy boggy lungs with hemorrhagic areas over lower lobes. Both adrenals were hemorrhagic. The brain, meninges, liver, spleen, heart, and gastrointestinal tract were unremarkable on gross.

On microscopy, skin showed fibrin thrombi in arterioles with acute inflammatory exudates and presence of Gram-negative diplococci within blood vessels [Figure 2]c. Bacterial colonies were also seen within pulmonary capillaries and hepatic arterioles and myocardium. Bone marrow showed hemophagocytic lymphohistiocytosis and presence of intracellular diplococci. Extensive proximal tubular necrosis and glomerular hemorrhages were seen. Cerebrospinal fluid was normal.

Antemortem blood cultures and nasopharyngeal swab culture confirmed the growth of  Neisseria More Details meningitides. Death was attributed to severe meningococcal sepsis with multiorgan failure and DIC.


   Discussion Top


Meningococcal infections are predominantly seen in developing countries. Asplenia, hereditary or acquired deficiencies of protein C and S, and complement deficiency predispose individuals to this infection.[3] Military recruits and children in daycare centers are at increased risk due to close contact and physical crowding. An important added factor in epidemiology is the carrier state. Acute infectious purpura fulminans have been reported from India earlier, but diagnosis following demonstration in peripheral blood and bone marrow has never been reported.[4],[5]

Our patient was a young immunocompetent male without any comorbidities. The only predisposing factor was physical crowding exposed to military recruit. At presentation, he was conscious and oriented without any meningeal signs. Like in this case, meningococcemia without meningitis may present as an undifferentiated clinical illness that may be difficult to diagnose. This is reported in only 5%–20% of patients with meningococcal disease.[6]

The appearance of a widespread petechial skin rash with fever was a pointer toward definite infectious etiology, and prompt therapy and appropriate investigations were initiated. Additional causes of vasculitis and broad range of differential diagnosis such as autoimmune disorders need to be excluded. In this case, circulatory collapse, shock, and multiorgan failure were pointers toward an infectious etiology and led to a fatal course. The peripheral smear examination was paramount, and diagnosis was confirmed on culture.

The pathogenesis of purpura fulminans has been linked to direct bacterial damage to the endothelium and DIC.[7]

Meningococci in culture are best isolated when incubated under carbon dioxide. The organism is fastidious in nature and inhibitors in blood culture bottle may prevent growth.


   Conclusion Top


This case highlights the distinct clinical presentation of meningococcal disease with purpura fulminans without meningeal involvement. Careful examination of peripheral blood smear may provide useful information before culture reports are made available.

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.
Darmstadt GL. Acute infectious purpura fulminans: Pathogenesis and medical management. Pediatr Dermatol 1998;15:169-83.  Back to cited text no. 1
    
2.
Smith OP, White B. Infectious purpura fulminans: Diagnosis and treatment. Br J Haematol 1999;104:202-7.  Back to cited text no. 2
    
3.
Biselli R, Fattorossi A, Matricardi PM, Nisini R, Stroffolini T, D'Amelio R, et al. Dramatic reduction of meningococcal meningitis among military recruits in Italy after introduction of specific vaccination. Vaccine 1993;11:578-81.  Back to cited text no. 3
    
4.
Gupta D, Chandrashekar L, Srinivas BH, Thappa DM. Acute infectious purpura fulminans caused by group A β-hemolytic streptococcus: An uncommon organism. Indian Dermatol Online J 2016;7:132-3.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Harikrishna J, Mohan A. Infectious purpura fulminans. Indian J Med Res 2015;141:130-1.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Poizeau F, Cormerais M, Darrieux L, Ricordel S, Garnier G, Safa G, et al. Meningococcemia without meningitis: A report of two cases. Rev Med Interne 2016;37:206-8.  Back to cited text no. 6
    
7.
Melican K, Michea Veloso P, Martin T, Bruneval P, Duménil G. Adhesion of neisseria meningitidis to dermal vessels leads to local vascular damage and purpura in a humanized mouse model. PLoS Pathog 2013;9:e1003139.  Back to cited text no. 7
    

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Correspondence Address:
Deepti Mutreja
Departments of Pathology, Command Hospital Air Force, Bengaluru - 560 007, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_209_17

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