Bone marrow manifestations in multicentric Castleman disease

This study aimed to document the morphological and immunophenotypic features, and describe the diagnostic features of bone marrow (BM) involvement in human herpes virus 8 Multicentric Castleman disease (HHV8‐MCD). BM trephine biopsy (BMTB) specimens from 28 patients were revisited. Samples were evaluated for expression of CD3, CD20, CD138, CD68R, glycophorin C, CD42b, HHV8‐latency‐associated nuclear antigen (LANA1), Epstein–Barr virus‐encoded small RNA and light chains. Presence of significant numbers of HHV8‐LANA1⁺ lymphoid/plasmacytic cells, noted in 10/28 cases, was indicative of BM involvement and was associated with low CD4 and CD8 counts in peripheral blood. The characteristic morphological appearance of MCD seen in lymph nodes is a rare finding in BMTB. 4/5 cases with lymphoid aggregates were involved by MCD, whereas 6/23 cases without lymphoid aggregates were involved by MCD (P = 0·023). 9/18 cases with hypercellular marrow were involved by MCD, whilst only 1/8 cases with normo/hypocellular marrow showed involvement by MCD (P = 0·070). While 9/21 cases with increased marrow reticulin were involved by MCD, none of the cases with no increase in reticulin were involved by MCD (P = 0·080). Reactive plasmacytosis is a frequent finding. We conclude that bone marrow is involved in a significant proportion of patients with MCD (36%), and involvement can be identified by HHV8‐LANA1 immunohistochemistry.     

Mechanical breakdown and thrombolysis in subacute massive pulmonary embolism: A prospective trial

AIM: To assess role of combined modality of mechanical fragmentation and intralesional thrombolysis in patients with massive pulmonary embolism presenting subacutely. METHODS: Eight of 70 patients presenting in tertiary care centre of North India with massive pulmonary embolism within 4 years had subacute presentation (symptom onset more than 2 wk). These patients were subjected to pulmonary angiography with intention to treat basis via mechanical breakdown and intra lesional thrombolysis. Mechanical breakdown of embolus was accomplished with 5-F multipurpose catheter to reestablish flow, followed by intralesional infusion of urokinase (4400 IU/kg over 10 min followed by 4400 IU/kg per hour over 24 h). RESULTS: Eight patients, mean age 47.77±12.20 years presented with subacute pulmonary embolism (mean duration of symptoms 2.4 wk). At presentation, mean heart rate, shock index, miller score and mean pulmonary pressures were 101.5±15.2/min, 0.995±0.156, 23.87±3.76 and 37.62±6.67 mmHg which reduced to 91.5±12.2/min (P=0.0325), 0.789±0.139 (P=0.0019), 5.87±1.73 (P=0.0000004) and 27.75±8.66 mmHg (P=0.0003) post procedurally. Mean BP improved from 80.00±3.09 mmHg to 90.58±9.13 mmHg (P=0.0100) post procedurally. Minor complications in the form of local hematoma-minor hematoma in 1 (12.5%), and pseudoaneurysm (due to femoral artery puncture) in 1 (12.5 %) patient were seen. At 30 d and 6 mo follow up survival rate was 100% and all the patients were asymptomatic and in New York Heart Association class 1. CONCLUSION: Combined modality of mechanical fragmentation and intralesional thrombolysis appears to be a promising alternative to high risk surgical procedures in patients with subacute massive pulmonary embolism.     

Primary mucormycosis of abdominal wall: A rare fungal infection in a immunocompetent patient

Mucormycosis of the anterior abdominal wall is an uncommon disease and it is very rare to find this disease in immunocompetent, non-diabetic patients which usually affects patients with trauma, with contaminated wounds, patients with underlying malignancies or patients with immunocompromised state, e.g., diabetics. We herein report a case of primary cutaneous mucormycosis in an immunocompetent and non-diabetic patient. Our patient was a 48-year-old female, executive by profession. She was diagnosed to have cutaneous mucormycosis of the anterior abdominal wall, and was managed with multiple debridements of the wound and intravenous amphotericin B therapy. She was administered a total of 1500 mg of liposomal amphotericin B and when fully healed, split skin grafting was done. We would like to emphasize the importance of high index of suspicion and early start of therapy in a condition with high rate of mortality.     

A novel finding of oligodontia and ankyloglossia in a 14‐year‐old with Floating‐Harbor syndrome

Floating‐Harbor syndrome (FHS) is a rare genetic condition characterized by distinct facial features, short stature and delayed skeletal development. Here we present case of a 14‐year‐old boy with short stature, typical facial features, impaired voice quality, clinodactyly, cryptorchidism and unilateral agenesis of kidney. In addition he had oligodontia and ankyloglossia with features suggestive of FHS. Treatment rendered was restoration of caries, application of pit and fissure sealants followed by frenectomy. The purpose of this report is to highlight the oral developmental anomalies and the management of a patient with FHS and to add to the current knowledge of the literature on this syndrome.     

A cost effective endovascular approach for management of post-catheterization profunda femoris artery pseudoaneurysm using thrombin

Post-catheterization PSA is one of the most commonly encountered vascular complications of cardiac and peripheral angiographic procedures. We report the case of patient who developed deep-seated profunda femoris artery pseudoaneurysm (PSA) following cardiac catheterization. Despite, repeated ultrasound guided compressions the PSA failed to close and instead produced local site pressure ulcers. The secondary infection followed which precluded use of percutaneous thrombin injection. The PSA was finally closed via a total endovascular technique combining intravascular thrombin injection and coil embolization, thus obviating the need for expensive measures like cover stents or invasive surgical repairs.     

Varied manifestations of Rosai-Dorfman disease and its management — a case report and review of literature

Rosai Dorfman disease or sinus histiocytosis is a rare, idiopathic, benign and self-limiting histiocytic proliferative disorder, usually seen in younger patients. It most commonly involves the cervical lymph nodes, with a predominant infiltration of sinusoidal histiocytes and classically presents with massive cervical lymhadenopathy. Extranodal disease occurs in about 43% of cases and produces different signs and symptoms depending upon its location. We report a case of this disorder in 22-year-old male with initial isolated involvement of bilateral nasal cavity and paranasal sinuses with subsequent involvement of cervical lymph nodes and skin. The clinical presentation, histologic characterstics, radiographic findings and treatment of the disease are discussed.     

Radiological diagnosis of the nasopharyngeal angiofibromas

Nasopharyngeal angiofibromas are notorious for silently extending to the regions adjoining their original sites. Their exact localization is essential for the total excision. Conventional X-rays have limited value in demonstrating extensions to base of skull, pterygopalatine fossa and paranasal sinuses. GAT Scanning when used along with contrast, provides clear delineation of every minute tumour extension, along with exact evidence of bone destruction. Angiography on the other hand is additionally useful in determining the vascular feeders, and differentiating from other regional tumefactions. Angiography may further clarify the vascularity and maturity of the tumours. The tumour blush in arterial phase suggests exact localisation of the lesion. Poor filling veins in venous phase indicate larger sinusoidal spaces. Mature or more fibrous tumours have better venous filling during this phase. These three modalities, therefore, appear to be complementary aids to the diagnosis and localisation of tumours and hence essential for planning the surgical procedure for total excision of these nasopharyngeal angiofibromas.