Prerna Mewawalla

Correction: Mantle Cell Lymphoma Relapsing as Disease of Skin, Orbit and CNS: An Extremely Rare Presentation and a Review of Literature

[This corrects the article DOI: 10.14740/jh363w.].

Aplastic anemia secondary to propylthiouracil: A rare and life-threatening adverse effect:

Background Propylthiouracil has been in use for more than half a century for the treatment of hyperthyroidism. While it is largely known to cause agranulocytosis, its association with aplastic anemia is rarely heard of. Our case will be the third in literature to suggest aplastic anemia as a manifestation of propylthiouracil, which unfortunately culminated in the death of the patient. Case A 67-year-old female, with recently diagnosed metastatic adenocarcinoma of the lung, developed hyperthyroidism after being started on Nivolumab and Iplimumab. After she developed atrial fibrillation, she was started on propylthiouracil to control the thyroid activity. Soon after that, she was admitted with severe neutropenia, which progressed to pancytopenia confirmed as aplastic anemia on a bone marrow biopsy. Despite discontinuation of propylthiouracil and aggressive treatment, she developed septic shock and multi-organ failure, leading to her death. Conclusion Aplastic anemia has been sparingly reported as an extremely rare complication of propylthiouracil. Further adding to the ambiguity is the unknown etiology and lack of specific therapy for the complication when attributed to propylthiouracil. The disease can carry an extremely poor prognosis if untreated, as proven by our case. Due to the same reasons, we recommend that further investigations be done to elucidate the pathogenesis and assist with treatment of the disease when caused by propylthiouracil.

Cholestatic jaundice: a unique presentation leading to the diagnosis of HLH with Hodgkin lymphoma, HIV and EBV

Haemophagocytic lymphohistiocytosis (HLH) is a syndrome of dysregulated immune activity with macrophage activation that can manifest as pancytopenia, coagulopathy and other laboratory abnormalities, usually progressing to multiorgan failure and death. This report documents the rarely reported association between HLH and Hodgkin’s lymphoma (HL) with simultaneous HIV and Epstein-Barr virus (EBV) and complete resolution with chemotherapy. The patient initially presented with cholestatic jaundice. He was then found to have HL associated with HLH with coexistent HIV and EBV viraemia. A-Brentuximab-VD regimen for the lymphoma was initiated, resulting in rapid resolution of HLH and ultimately remission of HL. HLH is a syndrome known to have high mortality; thus, early diagnosis and prompt treatment are crucial. Usual presentation includes non-specific symptoms and can easily be overlooked. This case highlights the importance of diagnosing the condition in unusual settings and attempting treatment by targeting the cause of HLH, HL in our case.

Hemophagocytic Lymphohistiocytosis Secondary to Bone Marrow Only B-Cell Lymphoma: A Very Rare Entity With an Even Rarer Presentation

Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome. It is categorized as familial or acquired, most commonly caused by infections, malignancies, rheumatologic and immunodeficiency disorders. Irrespective of the etiology, the age at the onset is the strongest prognostic factor, hence it is extremely important to have a high suspicion for HLH, diagnose it promptly and initiate treatment without any delay. We encountered a 70-year-old female patient who initially presented with left-sided facial weakness and pancytopenia, secondary to diffuse stage IV B diffuse large B-cell lymphoma with isolated bone marrow involvement with secondary HLH.

Toxic Hemolysis in a Young Healthy Female

Clostridium perfringes is a gram-positive, rod-shaped, anerobic, spore-forming pathogenic bacterium. C. perfringes is commonly known to cause tissue necrosis and gas gangrene. However, severe life-threatening complications like hemolysis due to clostridium sepsis are rarely encountered. We present a case of a 35-year-old female presenting with endometritis complicated with severe hemolysis in the setting of sepsis secondary to C. perfringes. Her hospital course was complicated with multi-organ involvement, requiring broad-spectrum intravenous antibiotics. Eventually, she made an uneventful recovery by early recognition of these rare complications and prompt institution of appropriate therapy.