Hyperdynamic State: bounding pulses, heart palpiations, raoring in the ears!
Acrocyanosis: dark, purple/gray discolartion of skin on most acral parts: finger tips, toes, nose, ears; changes disappear when warming; absence of paroysmal pallor; pain/discomfrot whne swallowing colf foods/liquids; painful
Difference between raynoauds: raynaud got sharply demarcated color changes of skin pallor followed by blue with rewarming, blushes (reactive hyperemia), looks for pins/ ndeedles, numbness, finger aching
Agglutination: Livedo Reticularis, cutaneous necrosis, urticaria
No tempeature related symptoms with my patient! Except for an inadeuqate response from infusion of blood products - > washed and warmed (washed to removed complememnt)
If sample cold, agglutination RBC may pass through counter in groups and will be counted as macrocytic cell!
If no reticu consider; iron def, b12, folate, coopper deficit, chornic disease?, etoh, bone marrow infiltrative disorder; marrow suppressive drugs
Shorter think about quantity/quality of antibody severity of disease
Can patient make reticulocytes?
Intravascular: pink or brown serum; dark urine; urine hemosiderin (prussian blue staining)
color changes in serum due to oxyhemoglobin/methemoglbinemia
Depending on pathway e.g. alternative pathway, classic, lectin 00> opsoniazation and phagocytosis vs directly lytics
High because present in everyone! But normally low e.g. < 1/64; so high is higher than 1/2000
Need all of these:
Hemolysis labs: Reticulocyte Count, Bilirubin, Haptoglobin, LDH, Smear, consider complment levels which c3/c4 should both be low
Why no spherocytes??
More than 70% will have one of these condiitions: (infection, AI, lymphoma)
Mycoplasma and Mono
RA, SLE
CLL, B cell NH lymphoma, Waldenstrom macroglobulinemia, monoclonal gammopathy of undetermined significance
% with lymphoma (high incidenee? 75%)
Best results to date have been obtained using rituximab alone or in combo
response rates as high as 75%
Rituximab along or in combo: other agents include bendmustine (appears to have best response rate), fludarabine, prednisone, interferon
Consider Steroid therapy if: patient with high thermal amplitutude, hemolysis at 37 celcius, IgG cold reacting antibodies, concurrent IgG warm agglutinins (IgG occurs when someone with mono treated with ampicillin)
Plasmapharesis: to reduce severe hemolysis, prepare patient for surgery, of acrocyanosis are severe,
Steroids: does not diminish antibody production, may downregulate phagocytosis; response rates are low maybe 14%)
Eculizumab blocks complmemnt
For Paroxysmal Cold: tx with support, transufions of warmed blood, avoid cold, consider presnidonse
PCH: due to cold reacting IgG generally after virus (varicella)
Cryoglobinulinema due to precipitation of blood proteins at low temperatures (hepatitis/HIV); antibodies are NOT reacting with RBCs);
Underlying condition: consider lymphoproliferative disease (especially if systemic systems like weight loss, night sweats fever, lymphadenopathy
Increase riks for VTE
Correct Answer: C
The most appropriate treatment for this patient is rituximab. She has profound anemia and evidence of intravascular (decreased haptoglobin, hemoglobinuria, elevated lactate dehydrogenase) and extravascular (elevated indirect hyperbilirubinemia level) hemolysis. The positive direct antiglobulin test showing C3 on erythrocytes with agglutinated erythrocytes on the peripheral blood smear suggests cold agglutinin autoimmune hemolytic anemia. The improbably high mean corpuscular volume (MCV) calculated by the Coulter counter likely reflects measurement of agglutinated cells, although the reticulocytosis will also elevate the MCV.
This disease can be primary, with no other underlying disorders, but may be associated with lymphoproliferative disorders, such as Waldenström macroglobulinemia, other B-cell non-Hodgkin lymphomas, and IgM monoclonal gammopathy of undetermined significance. Cold agglutinin disease may also be precipitated by infections, typically Mycoplasma pneumoniae or Epstein-Barr virus. In cold agglutinin disease, IgM antibodies are directed against erythrocyte antigens, resulting in complement fixation and intravascular hemolysis as well as clearance of complement-coated erythrocytes by Kupffer cells in the liver.
Rituximab, a monoclonal antibody to the B-cell antigen CD20, has shown efficacy in case series of patients with cold agglutinin disease. Additionally, all patients with cold agglutinin disease should be kept warm, and all infusions should be administered at body temperature. Unlike in patients with warm antibodies, prednisone and intravenous immune globulin do not decrease the hemolytic process in patients with cold agglutinin disease. Because cold agglutinin disease does not involve splenic clearance of erythrocytes, splenectomy is not effective at reducing this type of hemolysis compared with warm antibody-mediated hemolysis.
