What is DRESS Syndrome?

DRESS (drug reaction with eosinophilia and systemic symptoms) is a poorly understood syndrome that has an unknown prevalence in the pediatric population because it is often missed by doctors. 

Symptoms include fever, an itchy rash covering over half the body, enlarged lymph nodes, and inflammation of the liver. A blood test reveals increased white blood cells, "atypical lymphocytes" (abnormally large and irregular lymphocytes because they are infected with the virus), and elevated eosinophils. 

What causes it? There have been links to both herpesvirus (including oral and genital herpes, epstein barr virus and roseola virus) and a couple months after starting certain anti-seizure medications. Bad cases will involve >90% of the skin and affect multiple organs such as the kidneys, and there may be involvement of the mucosal lining of the mouth and lips which can mimic life threatening drug and infectious reactions known as Stevens Johnson Syndrome and Toxic Epidermal Necrolysis.

It is very difficult to avoid getting this syndrome, as it is unknown why some patients get it and others do not. It can take months to fully recover from DRESS syndrome. The treatment, if caused by a medication, is to stop that medication. Most patients get IV or topical steroids to calm the immune system. 

Most often, pediatric rashes are harmless. In rare cases such as this, however, some detective work is required to make a swift and proper diagnosis.

Death From Simple Infections: Disorders of Neutrophils

White blood cells (WBC) are made in our bone marrow and are absolutely imperative to fight infection. Without WBCs, we would succumb to even the most benign viruses and bacteria (i.e. germs) in our environment and die at a very early age. Once WBCs are made, they are released into the blood to mix with the RBCs (cells that carry oxygen) and circulate around our body. Stem cells in the bone marrow give rise to multiple WBC types, namely lymphocytes (T cells and B cells), neutrophils, basophils, eosinophils, and monocytes. Each type of WBC has a specific function. Eosinophils, for example, are important when fighting parasitic infectious. Many people are familiar with the Human Immunodeficiency virus (HIV), a disorder of T cells which impairs the body's ability to fight life-threatening bacterial and fungal infections. Neutrophils play a major function in our first line of defense against bacteria and fungi, and problems with them can carry grave consequences.

Neutrophils are typically first to the scene of an infection or traumatic injury. They are very quick, and their lifespan is only a few days. They follow chemical trails left behind by inflammation and, when they arrive, they suck bacteria into the center of their cell where some sanitizing then takes place- trapped bacteria are basically bathed in hydrogen peroxide. Two things can go wrong in this fight against the bacteria: either the neutrophils fail to work correctly, or the numbers of neutrophils are far too low to overpower invaders. Mildly low levels lead to oral/vaginal/rectal ulcers, ear infections, and skin abscesses. Severely low levels lead to overwhelming infection and death.

Disorders of the FUNCTION of neutrophils:
Congenital Leukocyte Adhesion Deficiency 1. Neutrophil movement is affected, so they are slow and fail to arrive to the scene of the infection. Newborn infants have delayed separation of their umbilical cord or infection where the cord meets the belly (omphalitis)

Chronic Granulomatous Disease. The release of the hydrogen peroxide inside the cell ("respiratory burst") doesn't work properly. Children need lifetime antibiotics (bactrim)

Hyperimmunoglobulin E Syndrome. The neutrophils don't follow the chemical trail to the site of infection like they are supposed to. Levels of an immune globulin called IgE are very high, and children have recurrent "cold" (non-red, non-tender) staph abscesses.

Chediak-Higashi Syndrome. There is a problem bringing the bacteria into the cell to sanitize them. Children also have albinism and their condition is worsened by infection with Epstein-Barr virus (EBV)


Disorders of the NUMBER of neutrophils:
External factors. Neutropenia caused by viruses (some viruses "shock" the stem cells and cause them to stop functioning for a period of time) or cancer chemotherapy

Autoimmune disorders. Neonatal Isoimmune Neutropenia occurs briefly in infants, whereby their mothers release antibodies that cross the placenta and attack their neutrophils. The disorder will resolve in less than 3 months. After 3 months, the baby can make their own antibodies against neutrophils leading to autoimmune neutropenia of infancy or chronic benign neutropenia.

Genetic syndromes. Shwachman-Diamond and Kostman Syndrome are characterized by low levels of neutrophils. Another interesting disorder is termed cyclic neutropenia, characterized by 3 day periods of neutropenia occurring every 21 days.

Deciphering Your Recent Blood Test, Part 1 of 3

On your recent visit to the doctor's office, you had the pleasure of getting stuck with a needle and drained of a few small tubes of your blood. You were left with a painful memory, a small bruise at the site of injection and a printout of the results. You spend a few seconds looking through the words and values before giving up, but wouldn't it be helpful if you really knew what all of those numbers meant? 

The laboratory blood test is one of the most useful diagnostic tools of the modern physician. It allows the provider to peer inside the body like a mechanic checks under the hood. There are many blood tests available, from single tests on proteins to "panels" containing several different measurements. Two commonly ordered panels are the complete blood count (CBC) and the comprehensive metabolic panel (CMP).

CBC

Our blood is made up primarily of red blood cells (RBC) and white blood cells (WBC). The RBCs carry nutrients through the body, while the WBCs protect us either by filling holes (platelets) or by attacking and killing whatever looks suspicious (leukocytes). The colors are red and white because, simply, when you spin the blood around very fast, these cells separate based on weight and reveal red-colored and white colored fluids. The CBC is performed by a machine that lines the blood cells up in a single row and counts how many are present in a set amount (microliter) of blood. 

WBC. An increase in WBC typically suggests that our body is training an army to kill an infection. This can also be elevated in cases of cancer or with the administration of steroid medications. The specific types of WBCs (neutrophils, eosinophils, basophils, monocytes or  lymphocytes) help give insight as to what type of infection may be present (for example, neutrophils are released acutely for bacterial infections). Decreases in WBCs are seen most commonly as a side effect of medications such as chemotherapy, antibiotics and anti-seizure drugs. In other cases, a decrease may suggest that something is killing them (infection, alcohol, autoimmune, radiation) or that there is a problem within the factory that produces them (the bone marrow). 

RBC and Hemoglobin. The primary role of the RBC is to carry oxygen, using hemoglobin, to cells so they can use it to make energy. When RBCs are decreased, it is known as anemia. This can be caused by iron (holds on to oxygen) deficiency, blood loss from chronic diseases, genetic diseases such as sickle cell anemia, significant bleeding or a problem with the factory that produces RBCs (the bone marrow). Increased RBCs can be seen in cases of dehydration, chronic breathing problems (COPD) and some cancers of the bone marrow.

Hematocrit. This is simply the ratio of red to white fluid when the blood is spun around very fast in a centrifuge. Normally, 46% of blood is RBCs. If this number is low, it's an anemia. 

MCV. This is the size of the RBC, much like measuring the square footage of a house. Increased MCV is seen with alcoholism, vitamin deficiency (B12 and/or folate) and a stomach disorder called pernicious anemia. Decreased MCV suggests one of the anemias described above.

MCH and MCHC. These measure the amount of hemoglobin per RBC, helping the physician narrow down the type of anemia present. MCHC is decreased in the anemias described above, and elevated in some genetic diseases.

RDW. This compares the RBCs to one another. If there is a large discrepancy between them, meaning some are very large and others are very small, this number will be high. The most common cause of this is iron deficiency anemia.

Platelets. Platelets are very important because they plug holes and cracks to stop bleeding. Low platelets are dangerous because it increases the risk of bleeding, whether out of a wound or into body cavities like the head or digestive system. High platelets are also dangerous because they cause over-clotting, which can lead to obstruction of blood vessels and thus problems like stroke and heart attack. Platelets are often kept intentionally low with medication (such as aspirin) to reduce the risk of stroke and heart attack. Many rare diseases and some cancers can also cause low platelet counts. 

• Parts 2 and 3 of this section, coming soon, will cover the CMP and other specialty tests.

• Your physician should discuss any abnormal blood test result with you. 

• Visit our resources at thedailydiagnosis.com for more trusted information on blood tests.

References

Chapter 5. Laboratory Diagnosis: Clinical Hematology. In: Gomella LG, Haist SA. eds. Clinician's Pocket Reference: The Scut Monkey, 11e. New York: McGraw-Hill; 2007. 

Klepin HD, Powell BL. Chapter 103. White Cell Disorders. In: Halter JB, Ouslander JG, Tinetti ME, Studenski S, High KP, Asthana S. eds. Hazzard's Geriatric Medicine and Gerontology, 6e. New York: McGraw-Hill; 2009. 

Verhovsek M, McFarlane A. Chapter 173. Abnormalities in Red Blood Cells. In: McKean SC, Ross JJ, Dressler DD, Brotman DJ, Ginsberg JS. eds. Principles and Practice of Hospital Medicine. New York: McGraw-Hill; 2012.