Angiosarcoma associated with Kasabach-Merritt syndrome presenting with typical signs and symptoms of pulmonary tuberculosis: a case report

Abstract

Background: Angiosarcoma is a rapidly proliferating vascular tumor that originates in endothelial cells of vessels. Rarely, it can be associated with consumptive coagulopathy due to disseminated intravascular coagulation eventually leading to thrombocytopenia and microangiopathic hemolytic anemia. This specific manifestation is termed Kasabach-Merritt syndrome. Patients usually present with manifestations related to the primary diagnosis of angiosarcoma depending on the organ it is involving. However, if Kasabach-Merritt syndrome has occurred, it will present with manifestations such as bleeding and thromboembolic phenomenon. To date, no favorable outcomes have been documented, and the overall prognosis remains grim.

Case presentation: A 44-year-old male patient of Afghan origin developed typical signs and symptoms of pulmonary tuberculosis, that is, fever, cough, hemoptysis, weight loss, and night sweats. He was initially managed in an Afghan medical facility where workup for tuberculosis was done but came back negative. He empirically received anti-tuberculous therapy owing to typical presentation and tuberculosis being endemic in the area. The condition of the patient worsened, and he presented to our facility (Shifa International Hospital, Islamabad, Pakistan). Workup led to the diagnosis of a metastatic vascular neoplasm, which was further complicated with consumptive coagulopathy, and microangiopathic hemolytic anemia. This presentation is known as Kasabach-Merritt syndrome. Multidisciplinary team discussion was called, and it was decided to proceed with palliative chemotherapy with paclitaxel.

Conclusion: Although a patient may present with typical signs and symptoms of, but negative workup for, TB, if there is a high index of suspicion and the patient is receiving empirical treatment for pulmonary tuberculosis, clinical worsening should alert to think about differential diagnosis. In our case, histopathological analysis of lymph node and radiological findings led us to the diagnosis.

Keywords: Angiosarcoma; Consumptive coagulopathy; Kasabach–Merritt syndrome; Microangiopathic hemolytic anemia (MAHA); Thrombocytopenia.

Fig. 1

Axial slices of contrast-enhanced CT of chest, mediastinal window showing enlarged subcarinal lymph node (a, blue arrow) and enlarged pre- and paratracheal and prevascular lymph nodes (b, red and blue arrows, respectively)

Fig. 2

a, b Axial slices of CT chest lung window showing innumerable, variable-sized, randomly distributed bilateral pulmonary nodules with the largest nodule containing internal calcification (blue arrow in b)

Fig. 3

a, b Axial slices of contrast enhanced CT showing a small hypodensity at the junction of segments VII and VIII (red arrow in a) and segment IVa (blue arrow in b)

Fig. 4

a, b Axial slices of CT bone window showing lytic expansile lesions with associated soft tissue components involving L5 vertebral body (blue arrow in a) and L2 vertebral body (blue arrow in b)

Fig. 5

Sagittal CT, bone window showing lytic lesions in L2 and L5

Fig. 6

Histopathology specimen of subcarinal lymph node

Fig. 7

Immunohistochemistry slide

Fig. 8

Bone scan with multi-level inhomogenous radiotracer uptake