Wednesday, December 5, 2007

Vera

A 60 year old man presents with an unusual chief complaint: he gets really itchy after he takes a bath. As you inquire further, you find other nonspecific complaints such as headache, weakness, dizziness, and excessive sweating. He also gets a weird sensation of burning pain in his hands and feet that's accompanied by erythema or cyanosis but he says that he feels a pulse when it happens. Past medical history is positive for peptic ulcer disease. On exam, you palpate a spleen and liver. You get back a CBC and well, all the numbers seem high. Here's a bone marrow biopsy stained for iron, 400x:


Challenge: What's the diagnosis?

Related Questions:
1. What's the symptom of itchiness called?
2. What's the symptom of burning pain called?
3. What's seen on the histology image?

Image shown under fair use.

2 comments:

Alex said...

1 histaminemia / pruritus
2 - algia?
3polycythemia rubra vera

exclude
"appropriate absoltue polycythemia" COPD, high altitude, hypoventilation syndromes like tetralogy, left shift Hb like methemoglobinemia

"absolute inapprorpriate polycythemia"
PRV, epo-secreting renal cyst, cushing's uterine leiomyomas, von hppel disease, hepatocellular carcionma

"relative polycythemia"
smoker's polycythemia, Gaisbock's syndrome, volume depletion

Craig said...

Vera

The symptom of itching is pruritis; the symptom of the burning pain in the hands and feet is erythromelalgia. The histology image shows a lack of iron in the bone marrow; iron has been used up to produce the increased red cell mass. Along with the CBC and history of peptic ulcer disease, this is highly suggestive of polycythemia vera. Polycythemia vera is a chronic myeloproliferative disorder with elevated red blood cell mass. Patients with polycythemia are prone to developing clots and further complications. It is related to a mutation in JAK2.

Sources: Tulane; UpToDate.
.
Wow, very thorough answer Alex!