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Vol. 99. Núm. 2.
Páginas 299-302 (1 março 2024)
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Visitas
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Vol. 99. Núm. 2.
Páginas 299-302 (1 março 2024)
Letter – Clinical
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Rapidly involuting congenital haemangioma (RICH) associated with transient thrombocytopenia and coagulopathy
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Ana María Palmaa,b, Tamara Gracia-Cazañaa,b,
Autor para correspondência
tamgracaz@gmail.com

Corresponding author.
, Carmen Ruiz de la Cuesta-Martínc, Yolanda Gilabertea,b
a Dermatology Service, Hospital Miguel Servet, Zaragoza, Spain
b IIS Aragon, Zaragoza University, Zaragoza, Spain
c Pediatric Service, Hospital Miguel Servet, Zaragoza, Spain
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Table 1. Summary of all cases published to date of RICH-type hemangioma and associated thrombocytopenia.
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Dear Editor,

A full-term male infant was born by natural delivery, with a vascular tumor in the right thigh of 10 × 5 cm in diameter, with central ulceration and no adhered to deep planes, since birth (Fig. 1). He was transferred to the neonatal intensive care unit at 3 hours of life after detecting hypoprothrombinemia (24% prothrombin activity), prothrombin time: 36.8 seconds (range: 9-12), no signs of hemolytic anemia, normal bilirubin, and normal platelet count, he was treated with vitamin K and 2 infusions of fresh frozen plasma. He presented with moderate thrombocytopenia (60 × 109/L) on the fourth day of life, which remitted along with rapid involution of the tumor. At 2 weeks of life, the tumor has completely resolved leaving a residual subcutaneous atrophy.

Figure 1.

10 × 9 cm vascular tumor, image taken at birth, necrotic ulcerated plaque is observed on the surface of the tumor.

(0.34MB).

He was treated from birth with prednisone 2 mg/kg/day for 5 days with withdrawal after improvement of the tumor. Given the clinical picture of a congenital vascular tumor with rapid involution, the diagnosis of rapidly involuting congenital hemangioma (RICH) was made, with no need for a biopsy.

With the diagnosis of RICH-associated coagulopathy, the patient has been followed up for 8 months with a very important regression of the lesion (Fig. 2).

Figure 2.

Progressive decrease in the volume of the tumor was seen 6 months of subsequent follow-up in consultation, disappearing ulceration with central necrosis and a persistent redundant skin plaque with residual surface telangiectasia.

(0.23MB).
Discussion

Coagulopathy and thrombocytopenia are complications that may appear in some vascular tumors, especially when they reach a large volume, due to thromboembolic complications and potential hemodynamic repercussions. The main differential diagnosis that should be considered in this patient is Kasabach-Merritt Syndrome (KMS), a life-threatening thrombocytopenic coagulopathy associated with rare vascular tumors, such as Kaposiform hemangioendothelioma, and less frequently with tufted angioma but not with common infantile or congenital hemangiomas.1

Unlike the persistent coagulopathy seen with KMS,1 the thrombocytopenia which appears in RICH commonly does not disturb coagulation factors or is not as pronounced, and then normalizes in the first month of life.2

In the literature published as RICH and transient thrombocytopenia, there are only 11 cases described, including the case that we have reported, all of them are summarized in Table 1.1,3–5

Table 1.

Summary of all cases published to date of RICH-type hemangioma and associated thrombocytopenia.

  Sex  Glut-1  Size  Location  Platelet  Start of thrombo-cytopenia  Resolution of thrombocytopenia  Clinical signs of regression of hemagioma  Treatment 
Baselga et al.1  Male  Negative  6 × 7 × 1.5 cm  Scalp  56 (×109 L–1Day 5  14 days  6 months (resected)  Surgical removal 
Baselga et al.1  Female  Negative  8 × 8 cm  Arm  62 (×109 L–1Day 4  14 days  14 days  Oral PDN for 4 days and embolization 
Baselga et al.1  Male  Negative  NA  Leg  5 (×109 L–1Day 2  7 days  5 days  Oral PDN, 1 month; platelet transfusion day 2 
Baselga et al.1  Male  NA  8 × 5 × 2.5 cm  Thorax  7 (×109 L–1Day 1  30 days  NA  Embolization 
Baselga et al.1  Male  NA  5.1 × 4.1 cm  Thigh  19 (×109 L–1Day 2  11 days  11 days  Intravenous dexamethasone, oral PDN 
Baselga et al.1  Female  NA  10 × 8 cm  Arm  21 (×109 L–1Day 8  60 days  2 months  Oral PDN, 2 months 
Baselga et al.1  Male  NA  11 × 13 cm  Leg  30 (×109 L–1Day 12  30 days  NA  Oral PDN, 2 months 
Braun et al.3  NA  NA  NA  NA  102 −108 g/L  Day 2-9  NA  NA  None 
Andreu-Barasoain et al.4  Male  NA  6 cm  Arm  34 (×109 L–1Day 16  1 month  1 year  Oral PDN for 1 month 
Rangwala et al.5  Female  NA  7.5 × 9.3 cm  Arm  3 (×109 L–1Day 10  2 week  1 month  A single platelet transfusion; Oral PDN for 5 days; Propranolol 1 mg/kg daily (1 month); flecainide; and a 6-week prednisolone taper. 
Palma et al.  Male  NA  10 × 5 cm  Thigh  60 (×109 L–1Day 4  2 weeks  2 weeks  Vitamin K and 2 infusions of fresh frozen plasma; Oral PDN for 5 days 

Thrombocytopenia in RICH may correlate with the size of the tumor because it has never been reported in RICHs of less than 5 cm, ranging up to 13 cm.

A male predominance was evident (sex ratio 2.3). The lesions were located in the extremities in 63.63% of the cases, which has already been previously demonstrated that the most common CH location was on the limbs. Regarding thrombocytopenia, in all of the patients it occurred in the first days of life, resolving in the majority of cases before 2 weeks. Most of them (72.72%) were treated with oral prednisone in the range of 2 mg kg daily with a minimum of 4 days and a maximum of 2 months. Finally, 100% of the lesions regressed spontaneously within the first year of life.

In conclusion, this association may be underreported and underdiagnosed since platelet count and coagulation studies are not routinely ordered in the evaluation of RICH.

Financial support

None declared.

Authors’ contributions

APR, TG, CCR and YG contributed to the preparation of manuscript and critically modified. APR and TGC contributed in the preparation of figures. All authors contributed to the article and approved the submitted version.

Conflict of interest

None declared.

References
[1]
E. Baselga, M.R. Cordisco, M. Garzon, M.T. Lee, A. Alomar, F. Blei, et al.
Rapidly involuting congenital haemangioma associated with transient thrombocytopenia and coagulopathy: a case series.
Br J Dermatol., 158 (2008), pp. 1363-1370
[2]
M. Sarkar, J.B. Mulliken, H.P. Kozakewich, R.L. Robertson, P.E. Burrows.
Thrombocytopenic coagulopathy (Kasabach–Merritt phenomenon) is associated with kaposiform hemangioendothelioma and not with common infantile hemangioma.
Plast Reconstr Surg., 100 (1997), pp. 1377-1386
[3]
V. Braun, S. Prey, C. Gurioli, F. Boralevi, A. Taieb, N. Grenier, et al.
Congenital haemangiomas: a single-centre retrospective review.
BMJ Paediatr Open., 4 (2020),
[4]
M. Andreu-Barasoain, E. Naz, M. Díaz, J.L. López-Estebaranz.
Rapidly involuting congenital hemangioma associated with transient anemia and thrombocytopenia.
Int J Dermatol., 52 (2013), pp. 1025-1026
[5]
S. Rangwala, A. Wysong, M.M. Tollefson, P. Khuu, L.T. Benjamin, A.L. Bruckner.
Rapidly involuting congenital hemangioma associated with profound, transient thrombocytopenia.
Pediatr Dermatol., 31 (2014), pp. 402-404

Study conducted at the Dermatology Service, Hospital Miguel Servet, Zaragoza, Spain.

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