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Leukemia cutis case

History: A 50-year-old woman presented with asymptomatic multiple cutaneous nodules all over the body of 4 months duration. Cutaneous examination showed multiple hyperpigmented nodules and plaques involving face, trunk, and extremities. Peripheral smear showed abnormally elevated leucocyte count (TLC-70,000) with abnormal cells: myeloblasts 40%, promyelocytes 8% and myelocytes 39%. Auer rods were present in few myeloblasts. What is the most likely diagnosis?

Gottron’s papules
Guttate psoriasis
Immune thrombocytopenia purpura (ITP)
Leukemia cutis
Solar purpura

Answer:

Leukemia cutis is the infiltration of neoplastic leukocytes or their precursors into the epidermis, the dermis, or the subcutis, resulting in clinically identifiable cutaneous lesions. Leukemia cutis may follow, precede or occur concomitantly with the diagnosis of systemic leukemia.

The clinical appearance of leukemia cutis is variable with erythematous to violaceous papules or nodules being the most frequent lesions (60%), followed by infiltrated plaques, generalised cutaneous eruption and erythroderma. They are frequently asymptomatic. The nodules are typically firm or rubbery in consistency, and can become purpuric if the patient is thrombocytopenic.

Source: PMC

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FDA approves Crizotinib for Myofibroblastic Tumors

The US FDA approves crizotinib (Xalkori) for the treatment of unresectable, recurrent, or refractory inflammatory anaplastic lymphoma kinase (ALK)-positive myofibroblastic tumors (IMT) in adults and children over 1 year of age.

Crizotinib is a selective tyrosine kinase inhibitor currently approved for the treatment of metastatic non-small cell lung cancer in patients whose tumors are positive for ALK or ROS1 as detected by an FDA-approved test, and for ALD-positive anaplastic large cell lymphoma.

Adverse reactions occurring in 35% or more pediatric patients included vomiting, nausea, diarrhea, abdominal pain, rash, vision disorder, upper respiratory tract infection, cough, pyrexia, musculoskeletal pain, fatigue, edema, constipation, and headache.

Source: Medscape

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Eruptive xanthomas case

History: A 30 yr old obese man presents with an asymptomatic rash on his back, arms, and hands that had developed 2 week earlier. On physical examination, scattered pink-yellow papules were present on the upper back, extensor surfaces of the upper arms, and dorsa of the hands. A fasting blood sample was grossly lipemic. Which of the following is the most likely diagnosis?

Eruptive xanthomas
Sebaceous hyperplasia
Granuloma annulare
Molluscum contagiosum

Answer: Eruptive xanthomas typically present as crops of 2–5 mm yellow papules with a red rim over extensor surfaces such as the buttocks or shoulders, but can be widespread including inside the mouth. The papules may be tender and are usually itchy. They may demonstrate köbnerisation. Eruptive xanthomas are due to hypertriglyceridaemia (triglyceride >11.2 mmol/L) of any cause.

Source: Dermanetz

Clinic Hours In FDA Updates

Dabrafenib Trametinib approved for metastatic tumors with BRAF V600E mutation

The US FDA has approved dabrafenib + trametinib for adult and pediatric patients aged 6 years and older with unresectable or metastatic solid tumors with the BRAF V600E mutation whose disease has progressed following prior treatment and who have no satisfactory alternative treatment options.

The combination represents “the first and only BRAF/MEK inhibitor to be approved with a tumor-agnostic indication for solid tumors carrying the BRAF V600E mutation, which drives tumor growth in more than 20 different tumor types, and it is the only BRAF/MEK inhibitor approved for use in pediatric patients.

The combination already carries indications for BRAF-mutated melanoma, non–small cell lung cancer, and thyroid cancer. The new approval was based on two adult trials and one pediatric trial that showed an overall response benefit for patients with high- and low-grade gliomas, biliary tract cancers, and certain gynecologic and gastrointestinal cancers.

Source: Medscape

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FDA approves Setmelanotide for obesity in Bardet-Biedl Syndrome

The US FDA has approved Setmelanotide, a melanocortin-4 receptor (MC4R) agonist, is the first FDA-approved therapy for BBS, a rare genetic disorder that impairs a hunger signal along the melanocortin-4 receptor (MC4R) pathway.

Individuals with Bardet-Biedl Syndrome typically have obesity that starts at age 1 along with insatiable hunger (hyperphagia). Available weight management options are generally unsuccessful. Other symptoms may include retinal degeneration, reduced kidney function, or extra digits of the hands or feet.

The most common adverse reactions (with an incidence ≥ 20%) included skin hyperpigmentation, injection site reactions, nausea, headache, diarrhea, abdominal pain, vomiting, depression, and spontaneous penile erection.

Source: Medscape

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Massive breakthrough as rectal cancer disappears in every patient in drug trial

Patients with locally advanced rectal cancer and tumors with deficient mismatch repair (dMMR) have shown a remarkable response to treatment with the programmed cell death-1 (PD-1) inhibitor dostarlimab (Jemperli).

So far, the study has involved only 12 patients, but all of them have had a clinical complete response to treatment. They continue to show no signs of cancer (during follow-up ranging from 6 to 25 months) and have not undergone surgery or had radiation and chemotherapy, which are the standard treatment approaches.

Dostarlimab is already approved by the US Food and Drug Administration for use in the treatment of recurrent or advanced endometrial cancer with dMMR. Rectal cancer is an off-label use.

All patients had mismatch repair-deficient stage 2 or 3 rectal adenocarcinoma. The authors note that these tumors respond poorly to standard chemotherapy regimens, including neoadjuvant chemotherapy. The median age of enrolled patients was 54 years and 62% were women.

Source: NEJM

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Madura foot case

History: A 50-year-old woman with painless swelling in her left foot. The swelling started after a banal penetrating injury on the sole of her left foot 23 years ago. X-rays images showed multiple osteolytic lesions of the tarsus.

a) Eumycetoma
b) Sporotrichosis
c) Chromoblastomycosis
d) Histoplasmosis
e) Lobomycosis

Answer:

Eumycetoma, also known as Madura foot.

Causes Madurella spp., Leptosphaeria senegalensis, Curvularia lunata, Pseudallescheria spp., Neotestudina rosatii, Acremonium spp. and Fusarium spp.

Symptoms: Swelling, weeping pus filled sinuses, deformity.

Diagnostic method Microscopy, biopsy, culture, medical imaging, ELISA, immunodiffusion, DNA sequencing

Differential diagnosis: Actinomycosis (Actinomycetoma)

Treatment Surgical debridement, antifungal medicines

Medication Itraconazole, posaconazole, voriconazole

Complications: Amputation

Prognosis Recurrence is common

Frequency Endemic in Africa, India and South America

Source: Wikipedia

Clinic Hours In FDA Updates

IN.PACT 018 Drug-Coated Balloon approved for PAD

The US FDA has approved the IN.PACT 018 drug-coated balloon (DCB) for the treatment of peripheral arterial disease.

The paclitaxel-coated balloon is indicated for percutaneous transluminal angioplasty of de novo, restenotic, or in-stent restenotic lesions up to 360 mm in length with vessel diameters of 4 to 7 mm, located in the superficial femoral and popliteal arteries.

The IN.PACT 018 DCB is built on the same technology as the IN.PACT Admiral DCB (0.035 inch guidewire compatible), but is engineered to cross tight lesions, provide better deliverability, and is 0.018 inch guidewire compatible.

Source: Medscape

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First Line for Esophageal Cancer is now Immunotherapy

Immunotherapy with nivolumab (Opdivo) is now approved in the United States for first-line use in the treatment of unresectable advanced or metastatic esophageal squamous cell carcinoma (ESCC).

The new approval for the drug, a programmed cell death ligand-1 (PD-L1) inhibitor, is for use in this patient population regardless of PD-L1 status. The indication also specifies that nivolumab is to be used together with chemotherapy (with a fluoropyrimidine- and platinum-containing regimen) or in combination with ipilimumab (Yervoy), an immunotherapy with a different mechanism of action.

Source: Medscape

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Rare monkeypox detected in UK: Check symptoms, treatment & other details

A patient was found to be infected with monkeypox virus in the United Kingdom, who traveled to the country from Nigeria.

Monkeypox is caused by monkeypox virus, a member of the Orthopoxvirus genus in the family Poxviridae. Monkeypox is a viral zoonotic disease that occurs primarily in tropical rainforest areas of Central and West Africa and is occasionally exported to other regions.

The incubation period (interval from infection to onset of symptoms) of monkeypox is usually from 6 to 13 days but can range from 5 to 21 days.

Monkeypox typically presents clinically with fever, rash and swollen lymph nodes.

Monkeypox virus is mostly transmitted to people from wild animals such as rodents and primates, but human-to-human transmission also occurs.
Monkeypox virus is transmitted from one person to another by contact with lesions, body fluids, respiratory droplets and contaminated materials such as bedding.

The clinical presentation of monkeypox resembles that of smallpox, a related orthopoxvirus infection which was declared eradicated worldwide in 1980.

Vaccinia vaccine used during the smallpox eradication programme was also protective against monkeypox. A new third generation vaccinia vaccine has now been approved for prevention of smallpox and monkeypox. Antiviral agents are also being developed.

Source: WHO