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  #1  
Old 07-26-2011
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Question Purple Hands upon cold exposure

A 72-year-old woman has had increasing fatigue with a 3 kg weight loss over the past 7 months. Her hands become purple and painful upon exposure to cold. On physical examination she has a palpable spleen tip. Laboratory studies show Hgb 10.5 g/dL, Hct 31.7%, MCV 99 fL, platelet count 193,600/microliter, and WBC count 5390/microliter. The direct Coombs test is positive at 4 C and negative at 37 C. Which of the following underlying diseases is this woman most likely to have?

A Non-Hodgkin lymphoma
B Systemic lupus erythematosus
C Pernicious anemia
D Scleroderma
E Thalassemia minor

Please Answer with Explanation
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  #2  
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D. Scleroderma

maybe part of the CREST syndrome underlying diseases? I would assume that she has Raynauds Phenomenon right now.
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I'd go with D.
The labs I attribute to cold hemolytic anemia, that's why we can palpate her spleen. The clinical presentation is consistent with Raynaud's. Also, look at the age of this lady, it is not very likely to have first attack of lupus at 73, non-hodgkin is also epidimiologically more common in younger people. Thalassemia is a microcytic anemia, pernicious is a macrocytic anemia.
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72y/o and a palpable spleen?...Non-Hodgkin lymphoma
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D. Scleroderma - Raynaud phenomenon with visceral findings
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it's D......CREST syndrome
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Quote:
Originally Posted by bebix View Post
72y/o and a palpable spleen?...Non-Hodgkin lymphoma
btw, secondary cryoglobulinemia:
- associated with lymphoproliferative disorder, autoimmune disease & infectious disease.

Since she has 72 y/o...the likelihood is greater for lymphoproliferative than autoimmune disease (more common: AR and SLE)
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Quote:
Originally Posted by bebix View Post
btw, secondary cryoglobulinemia:
- associated with lymphoproliferative disorder, autoimmune disease & infectious disease.

Since she has 72 y/o...the likelihood is greater for lymphoproliferative than autoimmune disease (more common: AR and SLE)
I dont know, I think "Her hands become purple and painful upon exposure to cold"

That's a clear cut indication of Raynauds, so I would choose D) Scleroderma aswell.

As others above explained, cold hemolytic anemia, dec. hct, speenomegaly all fit in.
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Quote:
Originally Posted by patelMD View Post
I dont know, I think "Her hands become purple and painful upon exposure to cold"

That's a clear cut indication of Raynauds, so I would choose D) Scleroderma aswell.

As others above explained, cold hemolytic anemia, dec. hct, speenomegaly all fit in.
Remember that Raynaud's phenomenon has a low specificity...

in this case she has secondary Raynaud's due to the presence of cryoglobulinemia...

People with cryoglobulinemia experience the characteristic symptoms - paleness, numbness, and pain in extremities = secondary Raynaud's...also, bleeding under the skin, and joint pain -- when exposed to cold weather.
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So, the patient has Raynaud...so, this could be A, B or D!!!

A Non-Hodgkin lymphoma - Both Hodgkin's disease and non-Hodgkin's lymphoma can occur in people of any age, but the risk of developing non-Hodgkin's lymphoma increases with age, with most patients being diagnosed in their 60s.

B Systemic lupus erythematosus - Sixty-five percent of patients with SLE have disease onset between the ages of 16 and 55

D Scleroderma - Peak age for the onset of scleroderma is mid-life between 40 to 50 years...this patient does not have a CREST syndrome (only has Raynaud)!!!...


So, just using epidemiological facts, I think the right answer is A)
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I think is A, She has cold autoimmune hemolytic anemia. Her RBC are being sequestred over the Spleen and producing Splenomegaly.

So Non-Hodgkin lymphoma should be considered.

Answer should be A
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Warning! A

A Non-Hodgkin lymphoma
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  #13  
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D- scleroderma
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Default Explanation

SOURCE : Webpath

(A) CORRECT. She has a cold autoimmune hemolytic anemia. Though this may be idiopathic or due to an infection (Epstein-Barr virus; Mycoplasma) a malignant lymphoma must be considered. Since the RBC's are being sequestered in the spleen with extravascular hemolysis, there is splenomegaly. A warm autoimmune hemolytic anemia will often produce intravascular hemolysis with hemoglobinemia that will more often lead to renal failure. There will more often be jaundice with intravascular hemolysis. Intravascular hemolysis will decrease haptoglobin more.

(B) Incorrect. SLE is more likely to be associated with a warm immunohemolytic anemia. SLE is less common with advancing older age.

(C) Incorrect. Pernicious anemia should be associated with a higher MCV (this woman's MCV could be explained by reticulocytosis). Megaloblastic anemias are not associated with immune hemolysis.

(D) Incorrect. Scleroderma can be associated with Raynaud phenomenon, but it is unlikely to be associated with an immunohemolytic anemia.

(E) Incorrect. Thalassemias can have ineffective erythropoiesis, but not immune-mediated hemolysis.
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  #15  
Old 09-15-2011
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Warning!

A is correct.

Old age
Raynaud
Splenomegaly
Anemia
Weight loss
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  #16  
Old 09-16-2011
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Warning!

I see some confusion here

this is not Raynaud phenomenon, this is hemmaglutination. these are 2 different phenomenon with different presentation.
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