Pulmonary Mucormycosis in Chronic Lymphocytic Leukemia and Neutropenia.

Pulmonary mucormycosis is a rare life-threatening fungal infection associated with high mortality. We present the case of a 61-year-old man with history of chronic lymphocytic leukemia who presented with fever and cough, eventually diagnosed with pulmonary mucormycosis after right lung video-assisted thoracoscopic surgery. The patient was successfully treated with amphotericin B and right lung pneumonectomy; however, he later died from left lung pneumonia

Atraumatic splenic rupture associated with apixaban.

Apixaban is a direct oral anticoagulant that works by inhibiting factor Xa. It has been associated with adverse bleeding outcomes including atraumatic splenic rupture. We present the case of an 86-year-old man who presented with features of left upper abdominal pain and hemorrhagic shock found to have atraumatic splenic rupture and hemoperitoneum on imaging

Acquired von Willebrand disease associated with monoclonal gammopathy of unknown significance

We present a case of a 79-year-old male who presented with retroperitoneal hematoma a week after motor vehicle accident. Prior history and family history of bleeding were nonsignificant. His activated partial thromboplastin time was found to be prolonged in the emergency department. Further workup with coagulation studies showed decreased factor VIII, vWF antigen, and vWF:ristocetin cofactor assay, and negative Bethesda assay, indicating acquired von Willebrand disease. Immunofluorescence to find an underlying etiology was suggestive of MGUS. Management of AvWD depends on controlling active bleeding and treating the underlying cause. He was treated with factor VIII, haemate-p, rituximab, two cycles of IVIg, and three weeks of oral steroids

AGEP overlap induced by hydroxychloroquine: a case report and literature review.

Acute Generalized Exanthematous Pustulosis (AGEP) is a rare drug reaction manifesting as pustular lesions with surrounding erythema following exposure. The disease is often self-limited and treatment is supportive. It may present in an atypical variant with vesicles that desquamate into erosions, which classifies the disease as an AGEP/SJS Overlap. This overlap syndrome can carry a substantial mortality rate and necessitate elevation in the level of care. Hydroxychloroquine has been implicated in cases of AGEP, and we present a case of AGEP/SJS overlap attributed to this common medication. Given the prevalence of drug eruptions, it is critical for the physicians to recognize and not overlook this rare and potentially fatal dermatological emergency

Acquired angioedema masquerading as abdominal and joint pain: c1 esterase deficiency secondary to cryptic lymphoma

A 62 year-old man presented to the emergency room with recurrent, colicky generalized abdominal pain. He had presented with similar abdominal pain in the preceding 6 weeks, requiring two hospital admissions. Admissions were accompanied by asymmetric lower limb swelling and pain on one admission, and swelling of lips on this admission. Six weeks prior, a computed tomography scan of the abdomen identified mesenteric lymph nodes which were biopsied and found to have CD5- positive cells consistent with chronic lymphocytic leukemia (CLL). The patient denied associated difficulty swallowing or breathing. He had no previous history of angioedema, food allergies or new medications. Vital signs were normal and physical examination was remarkable for symmetric upper and lower lip swelling without hives. Given lip swelling and recently diagnosed lymphoma, acquired angioedema was suspected. C1 esterase deficiency was confirmed by low functional C1 esterase inhibitor level, (4%, normal: \u3e 40%), low C1 esterase inhibitor antigen (/dl, normal: 21 – 39 mg/dl), low C4 complement (\u3c 1.7 mg/dl, normal: 12 – 38 mg/dl), and low normal C1q level (113ug/ml, normal: 109 – 242 ug/ml). Sedimentation rate, C-reactive protein, CBC and liver functions were normal. Patient was given one dose of intravenous Methylprednisolone 125 mg and antihistamines emergently. He was subsequently commenced on C1 esterase replacement, Icatibant (a bradykinin B2 receptor antagonist) and chemotherapy for lymphoma. He has had no further episodes of abdominal pain or lip swelling. An epinephrine pen was made available to him should angioedema recur Discussion: Angioedema is easily recognized clinically as facial or lip swelling, airway obstruction with or without hives. Lymphatic malignancies are a recognised cause of rare C1 esterase deficiency leading to acquired angioedema. Abdominal pain and extremity pain or swelling in isolation can be the only symptom of acquired angioedema. Conclusions: Clinicians should be aware of angioedema presenting with abdominal and extremity pain/swelling without lip swelling. Older patients presenting with new angioedema should prompt a consideration of acquired C1 esterase deficiency with suspicion for underlying malignancy especially lymphoma, adenocarcinoma, monoclonal gammopathy of undetermined significance, and less commonly autoimmune diseases